Hematology Flashcards

Terms Definitions
Microcytic AnemiaNormal Iron StudiesNormal RBC and FHx of anemiaWhat are thinking?
Thalassemia
Quantitative defect in hemoglobin:
Thalassemia
Where is folate absorbed?
jejunum
Reticulocyte count is representative of bone marrow function 1. RBC production increased, Retic count increases 2. RBC production decreases, Retic count decreases
What food contains B12?
meat only
How to calculate Fe saturation?
serum Fe/TIBC
Erythropoietin made in the kidneys drive RBC production, you generate more erythropoeitin due to hypoxia (anemia> carry less oxygen> hypoxia> erythropoietin secreted from kidneys> make more RBCs).
Anemia history:
evidence of recent bleedinghealth changes recentlymedication changeschanges in energy level, fatigue
Treat pernicious anemia: take extra amount of B12 to overcompensate
What type of hemolysis is PNH?
intravascular
This causes intra- or extra-vascular hemolysis?

- mechanical injury
defective cardiac valves
thrombi
intravascular
Cold or warm immunolytic anemia?

- ears, fingers, toes mostly affected
- vascular obstruction (pallor, cyanosis)
cold
Cold or warm immunolytic anemia?

- extravascular hemolysis
- mildly enlarged spleen
warm
Microcytic algorhythm:
After diagnosis of microcytic anemia check: 1. Iron studies: low ferritin and high RDW> iron deficiency anemia2. Check RBC count- high RBC, low RDW: Thalassemia- low RBC> sideroblastic or anemia of chronic disease(sideroblastic anemia tends to have normal iron studies. anemia of chronic disease will have low serum iron and transferrin and TIBC, but high ferritin)
Beta thalassemia minor:
one beta gene, increased HbA2/HbF
With thalassemia the RBC count is normal to increased b/c __> erythropoeitin released> increased RBCs.
hypoxia
Thalassemia all iron is normal, RBCs are normal to high. Sideroblastic anemia, all iron is normal, RBcs are low. Anemia of chronic disease, serum iron is low, transferrin is low, TIBC is low, and ferritin is normal to high.
Example of decreased production causing anemia:
bone marrow failure
Most common cause of macrocytic anemia:
B12/folate deficiencies
Most common cause of microcytic anemia:
iron deficiency
Which form of folate enters circulation?
5-methyl FH4
What is this disease?

- hypochromatic, microcytic
- koilonychia, cheilosis
- PICA
iron deficiency anemia
What is this disease? be specific.

- morphology:
target cells
Hgb crystals
HbSC
What drugs can cause megaloblastic anemia?
cancer drugs
Defects in either the alpha or beta chains of hemoglobin, leading to ineffective erythropoiesis and hemolysis
Thalassemia
Distribution of thalassemia parallels that of __ __.
Plasmodium falciparum (malaria): Mediterranean, Africa, Western and SE AsiaIndia, Burma
Both b12 and folate deficient anemias are macrocytic and have __ __.
hypersegmented neutrophils
Extrinsic Hemolytic Anemia:
- drug reactions- prosthetic heart valves (mechanical)
Microcytic anemiaLow iron levelsLow transferrinDecreased TIBCNormal to increased ferritin
anemia of chronic disease
Treatment of hereditary spherocytosis:
- splenectomy - folate replacement
ringed sideroblasts are seen with what type of anemia?
sideroblastic anemia
What if there is microcytic anemia, but the iron studies are normal? What is the next step?
RBC count
For females, a Hemoglobin less than __ is considered anemia.
12
Sickle cell anemia symptoms:
- fatigue- susceptibility to infection- delayed growth and development in children- episodes of severe pain, especially in joints, abdomen, and limbs
What is the most reliable diagnostic test for sickle cell anemia?
Hb electropheresis
What are these symmptoms indicative of?

- koilonychia
- cheilosis
- PICA
- alopecia
- atrophic glossitis
iron deficiency anemia
Cold or warm immunolytic anemia?

- intermittent intravascular hemolysis after exposure to cold
- increase indirect bilirubin
paroxysmal cold hemoglobinuria
What is this?

- decrease Fe+, ferritin
- increase transferrin
iron deficiency anemia
Chronic blood loss usually manifest as ____.
iron deficiency
beta thalassemia major:
2 beta genes, severe, failure to thrive, sig HbF
The only anemia that is hyperchromic:
Hereditary spherocytosis> normocytic hemolytic hyperchromic anemia
The end result of sideroblastic anemia is ineffective ___. The iron enters the developing RBC but ___ in the mitochrondria b/c it cannot be used for hemoglobin synthesis. This gives the nucleus a ringed appearance.
- erythropoiesis- accumulates
What is the most likely cause of iron defiency anemia in women before manopause?
menses
What is this disease?

- dyspnea on exertion
- impaired catecholamine and T3 metabolism
- hemosiderin in BM
iron deficiency anemia
After acute blood loss, when does bone marrow reponse peak during restoration process?
7 days
What is this disease?

- high levels of homocysteine, methylmalonic acid
- positive schilling test
Vit B12 deficiency
An asian couple with alpha-thalassemia trait is considering having a child. What should you worry about?
HbH in the child
Disease states with decreased reticulocyte counts:
- Aplastic anemia- Iron deficiency anemia before treatment- Pernicious anemia before treatment
Acquired causes of sideroblastic anemia:
- drugs- LEAD EXPOSURE- collagen vascular disease- neoplastic disease
Sideroblastic anemia is caused by an abnormality in RBC __ __. It can be __ or __.
iron metabolismgenetic or aquired
Hereditary spherocytosis is an intrinsic cause of mild__ __ anemia. It is the only anemia that is ___. It is an __ __ disease, thus there will be a family history.
- mild normocytic hemolytic anemia- hyperchromic- autosomal dominant-
What are some myelotoxins that cause aplastic anemia at high doses?
- benzene
- chloramphenicol
- alkylating agents
- antimotabolites
What is this disease?

- glycolytic pathway abnormality
- AR transmission
- reticulocytes and poikilocytes
PK deficiency: chronic hemolytic anemia.
What is schilling test?
Give PO radioactive B12 and measure urine levels to see if they are absorbed. If not, give IF to see if it corrects it. If it does, the patient is IF deficient.
What diseases are protective against malaria?
- G6PD
- HbS
- alpha thalassemia
Explain the restoration process after acute blood loss.
- leukocytosis: granulocyte mobilized from marginal pool
- water shift from interstitial fluid compartment
- low Hct, oxygenation
- EPO release (kidney)
- CFU-E proliferation (bone marrow)
How do you treat HS and HE?
Splenectomy after age 5.
Possible labs for anemia workup:
- CBC w/ diff- Peripheral smear- Reticulocyte count- Iron studies- B12- Folate
signs of lead poisoning:
- lead line on gums and epiphyses- encephalopathy- erythrocyte basophillic stippling- wrist and foot dropsfirst line tmt: Dimercaprol and EDTA
main difference b/w labs of thalassemia and sideroblastic anemia:
- Thalassemia: normal or increased RBCs- Sideroblastic anemia: low RBCs
How do you check for hemolsyis?
look for LDH (lactic acid dehydrogenase)
With anemia of chronic disease there is often a mild __ component. Erythropoietin levels are normal to high, but still low considering level of anemia. This is a diagnosis of exclusion. Must first rule out __ __.
- hemolytic - iron deficiency
What is a specific diagnostic test for pernicious anemia?
Serum test for anti-IF.
List some causes of microangiopathic hemolytic anemia.
- cardiac valve prosthesis
- malignant HTN
- SLE
- TTP
- HUS
What diseases may have atrophic glossitis?
- B12 deficiency
- iron deficiency anemia
- anemia of chronic diseases
- plummer-vinson syndrome
What are some chronic diseases that cause anemia?
- chronic microbial infection
- chronic renal failure
- chronic immune disorder
rheumatoid arthritis
enteritis
- neoplasm
Hodgkin's lymphoma
lung cancer
What is the most common cause of anemia among hospitalized patients?
anemia of chronic disease
How does human body compensate anemia?
- increase cardiac output
- shift O2-Hb dissociation curve
- increase EPO: increase reticulocytes
- bone marrow expansion into fatty spaces, may cause bone deformity if longterm and if in young kids.
What is the function of hepcidin?
Hepcidin: inhibit iron uptake and release from macrophages
What should you give HbS patient prophylactively? and for what reason?
Penicillin for infection with encapsulated organism because of autosplenectomy and impaired alternative complement pathway(impaired opsinization).
What are some complications of beta-thalassemia major?
- iron overload -> cardiac disease
- hemosiderosis, 2nd hemochromatosis
- prominent facial bones, sparing of the mandible.
What is the genetic difference in pathogenesis of alpha and beta thalassemia?
alpha thalassemia: deletion on chromosome 16

beta thalassemia: mutation (most commonly splicing sites) on chromosome 11
What is the treatment for sickle cell anemia? and what is the drug mechanism?
Hydroxyuria:
- increases HbF
- anti-inflammatory
- increase RBC volume
- oxidized by heme to produce NO
Symptoms of anemia caused by sudden RBC destruction:
- abdominal pain- red/brown urine- jaundice- small bruises under the skin- seizures- kidney failure symptoms
pregnancy would likley result in what type of anemia:
iron deficiency b/c of increased demand
Sickle cell is __ __ and results in __ __ anemia. 10% of African Americans have the heterozygous trait.
autosomal recessivenormocytic hemolytic
Symptoms of sideroblastic anemia caused by lead poisoning:
- blue-black line on gums known as lead line- abdominal pain- constipation- vomiting
What is this disease?

- intrauterine fetal death
- Hb bart
hydrop fetalis: 4 alpha globin gene deletion.
Why do patients with HbS have screw-cut skull or prominent cheek bones?
bone marrow hyperplasia -> bone resorption -> 2nd bone formation
What diseases/conditions would you think of when you see Heinz bodies on a blood smear?

How to differentiate them apart?
- G6PD: will also see bite cells
- lead poisoning: decreased ALA synthase and dehydrase in Hgb synthesis -> will also see ribosome aggregation.
What types of anti-IF are there for pernicious anemia and what are the predominance of each type?
Type I(75%): anti-IF
Type II(50%): block B12-IF from binding to ileal receptors
Type III(90%): target gastric pump
How would alcolics develop foate deficiency?
- traps folate in the liver
- excessive urinary loss
- disordered folate synthesis
What are some non-congenital cause of pure red cell aplasia?
- immune mechanism
- 50% associated with thymoma
- large granular lymphocytic leukemia
- drugs
What will iron indices show in sideroblastic anemia?
Iron overload: - increased serum iron - increased ferritin- normal TIBC- normal RDW
Lab values for B12 deficiency: KNOW THIS
- Cobalamin less than 200 pg/ml- elevated serum methylmalonic acid- elevated homocysteine
Anemia with MCV b/w 81 and 100 fl. Reticulocyte count is increased. Lactic acid dehydrogenase(LDH) is not found. Whats going on?
Normocytic> increased retics means bone marrow is responding> lack of LDH means there is no hemolysis> RECENT BLEED RESULTING IN NORMOCYTIC ANEMIA
Why is EPO low in anemia of chronic disase?
chronic disease -> IL1,TNF,IFN -> reduced EPO from kidney
What is the function of R-binder? Where is it come from?
R-binder
- bind to B12 in the stomache
- from salivary gland
Where is B12 absorbed in the intestines?
Ilium: B12-IF bind to receptors on ileum.
In general, anemia presents with what symptoms:
- easy fatigue and loss of energy- rapid heart beat, especially with exercise- SOB and headache, especially with exercise- difficulty concentrating- dizziness- pale skin- insomnia
Folate is normally absorbed in the ___ and __ __. Deficiencies are seen with __ __, ___, __, __ __ __, and ___.
- dudodenum - proximal jejunum - celiac disease- amyloidosis- pregnancy- sickle cell anemia- alcoholics
With anemia of chronic disease, iron cannot be transferred into the plasma, so the stores are high. Based off of this describe an iron panel of a patient with anemia of chronic disease:
- low serum iron- low transferrin- low TIBC- normal to increased ferritin
What will you see in the bone marrow of a B12 deficient patient?
- marrow hyperplasia with decreased M/E ratio
- hypersegmented granulocytes
- megaloblastic change
What is the underlying mechanism of B12 deficiency?
Lack of B12 traps N5-methyl FH4 which is essential for DTMP synthesis which is needed for thymine synthesis.
Anemia with MCV between 81 and 100 fl. Reticulocyte count is increased. Lactic acid dehydrogenase and Indirect unconjugated bilirubin is detected. Whats going on?
- Normocytic, increased retic so bone marrow is responding, presence of LDH indicates> HEMOLYTIC ANEMIA
Where is B12 stored and for how long?
B12 is stored in the liver for yrs.
What is the function of IF? Where is it come from?
IF:
- bind B12 in the duodenum
- made by the parietal cels
-cyte
cell
-poietin
stimulates production
Sp
Serum Protein
Bone marrow examination
MCV calculation
Hematocrit/RBC count
Stem Cell immuno markers
CD34+
Starry sky pattern
Burkits lymphoma
Name the stain.
Specific esterase
Endemic Burkitt's
Africa
Jaw disease
EBV related
Thrombocytopenia Complications
Hemorrhage
Cerebral hemorrhage fatal
Polycythemia Vera
Proliferation of erythroid, granulocytic and megakaryocytic elements; Absolute increase in RBC mass (becuase the dominant cell line affected are the erythroid precursors); Decreased erythropoietin (because there are so many RBCs present, EPO production isn't triggered); Jak-2 mutation (not specific for P.vera, but it is detected in 90-95% of p.vera cases)
Genetic abnormality w CML
"t(9,22) Philly"
Antithrombin
natural anticoagulant. Inactivates factors IXa and Xa and thrombin
calculating MCV
MCV= Hct X 10/RBC
What blood cell?
Professional APC's
Dendritic cell
Alcoholic blood smear
Macrocytic
Ovalocyte, hypersegmented PMNs
Acanthocytes
Target cells
Glanzmann thrombasenia
Deficiency in GPIIb/IIIa receptor

Bleeding disorder
Post transfusion purpura
Reaction against platlets
WBC
Total white blood cell count
neutrophils
WBC, granulocyte; function as phagocytes to ingest and destroy small particles, stress can increase production; increased at birth but decrease during the first week
What is a Normal Hemoglobin?
14-18 mg/dL
Calculation for WBC estimate
Avg x 3000
Reed Sternberg immuno markers
"CD 10-,20-15+,30+, LCA-"
Fibrin degradation product that is very sensitive (but not specific) for thrombis
D-Dimer
Myeloblast
The first recognizable "mother cell" (precursor) of the granulocytic cell line.
Leukocytosis
Increased WBC's -infection and or inflammation -increased bands - immature neutrophils, severe infection, used up all neutrophils
What type of anemia?
Sideroblastic anemia
Microcytic anemia
Describe multiple myeloma.
-possible neutropenia
-rare abnormal circulating plasma cell
-normocytic/normochromic anemia
-rouleaux
-platelet count normal to decreased
Neutropenia define
Too few white blood cells
Primary myelofibrosis

Treatment
Stem cell transplant is curative

Hydoxyurea, transfusions, splenectomy
Secondary lymphoid follicle
Antigenic stimulation for differentiation
Creates germinal center with follicular dividing cells
Surrounded by mantle of transient small lymphocyytes
CLL presentation
Asymptomatic
Male (2:1)
> 50 (median is 65)
Lymphocytosis with small mature lymphocytes
or
Adenopathy
Auer rod
malformed azurophilic granules; can occur in myeloblast
What is Leukocytosis
An increase in Leukocytes
increase deformability
spherocytes differ from normal cells
Platelets
term > preterm; < 150,000= abnormal; source: megakaryocytes; life span: 10 days; size increases with gestational age; stimulated by thrombopoietin
During coagulation phase 3, thrombin catalyzes the polymerization of ____ into _____.
fribrinogen, fibrin
Etiology of Multiple Myeloma
Unknown; Suggested predisposing factors include: viral infection with human herpesvirus 8 (HHV-8), MGUS (monoclonal gammopathy of undetermined significance)
Essential Thrombocythemia
The least common of the myeloproliferative disorders; Megakaryocyte proliferations; Increased platelets (thrombocytosis); Thrombosis; Hemorrhage
What is hemophilia A?
A Factor VIII deficiency
Tx for vWD
Desmopressin (DDAVP) which induces release of vWF
What is the anemia d/t impaired erythroid precursor cell division/DNA synthesis?
Macrocytic
Erythrocyte
A mature red blood cell or corpuscle.
function of G-CSF
primarily, but not exclusively, stimulates production of granulocytes
MCV
The average size (volume) of an RBCNormal values: 80-100 fl. (a femtoliter [fl] = 10-15 liter)
condition caused by extensive sickling, which is where sickle cells block blood flow and cause tissue death

causes pain in the chest, back, and extremities
sickle cell crisis
This RBC form indicates?
Spherocyte
Hereditary spherocytosis, autoimmune hemolysis
MCH
the average hemoglobin content of a RBC.

Normal values: 26-34 pg. (a picogram [pg] = 10-12 gram)
What disease states are associated with pappenheimer bodies?
-splenectomy
-hemolytic anemia
-sideroblastic anemia
-megaloblastic anemia
-hemoglobinopathies
What is dificient in Hemophilia A?
Factor VIII
Fibrinogen in thrombus formation
Bridging protein platelet aggregation
Lead poisoning symptoms
Anemia
Lead line in gums
Motor neuropathy -- lead palsy
Autonomic neuropathy -- ileus/abdominal pain - lead colic
New Methylene Blue
supravital stain; (another is Brilliant Cresyl blue stain) stains RNA- shows reticulocytes, and also hemoglobin - Heinz bodies
Neutropenia Nursing Management Safety
Avoid exposure/risk for infection
Strict hand washing
Avoid invasive procedures
Pulmonary hygiene (IS, TCDB)
What is the most plentiful plasma protein?
Albumin
Spherocytes differ from normal cells in what ways?
Increased deformability
Thrombocytopenia- pos causes
chronic hypoxia, asphyxia, DIC, sepsis, congenital viral infections, NEC, MAS, PPHM, pre-eclampsia. immune mediated maternal antibodies
What are some s/sx of HDNF?
Hepatosplenomegaly, jaundice, anemia
WBC, RBC, HGB, HCT, PLT decreased
MCV, MCH, RDW increased
hyper-segmented neutrophils
Howell-Jolly bodies
decreased folic acid or B12
megaloblastic anemia
Adrenal Glands and Lymphoma
Adrenal involvement is rare; Non-Hodgkin's > Hodgkin's; 4% of Non-Hodgkin's patients; 50% bilateral; May present with adrenal insufficiency; Non-specific imaging appearance (U/L or B/L adrenal enlargement, associated with retroperitoneal adenopathy, other extranodal sites)
G6PD A-
An abnormal genetic variant of G6PD that is found in 10% of American blacks; The enzyme is synthesized in normal amounts, but is unstable with a short half-life resulting in a marked deficiency in (and therefore hemolysis of) older red cells
B-Cell Antigen-Dependent Development
Germinal Center Reaction (the purpose of which is to produce antigen-specific plasma cells); Plasma cells of the bone marrow; Memory cells are generated; Tumors of these types of cells are more prevalent in adults
What are the medications that can cause thrombocytopathia?
aspririn, NSAID's
"Point mutation often seen with PV, ET, MF"
Jak2 gene
Where does the common pathway start
With Factor X
Papenheimer's Bodies
Basophilic inclusions in the red blood cells that is cluster like. They are believed to be iron particles confirmation is made by Prussian blue stain.
Pancytopenia
A depression of each of the normal bone marrow elements: white cells, red cells, and platelets in the peripheral blood.
Atrophy
decrease in number and size of cless that can lead to decrease in organ size or tissue mass
Lymphocytosis
Mononucleosis - caused by EBV - assoc w/lymphoma -> affects B lymphocytes - infected cells proliferate and form heterophile antibodies (diagnosis) Generalized biral symptoms, sore throat, malaise, anorexia, enlarged lymph nodes Complications - encephalitis, Guillain-barre = ascending paralysis, peripheral to central, resp sys, up to years to progress
In which type of anemia do the symptoms depend on the number of genes involved?

(If one gene is involved, it is considered minor and person is usually asymptomatic.

If 2 genes are involved, it is considered major and person can become quite ill).
thalassemia
What factors make up the intrinsic pathway?
XII▶XI▶IX▶X(Common pathway)
differential diagnosis for pancytopenia
marrow --> aplastic anemia, metastatic cancer to marrow, myelofibrosis
infectious --> parvirus, retrovirus
auto-immune --> B12 deficiency, SLE, hypersplenism
drugs --> chloramphenicol
acute leukemia is only one that shows blasts in peripheral blood
Describe vacuoles in leukocytes.
Unstained circular area; usually within cytoplasm; few to many.
What are the functions of PMNs?
Chemotaxis
Phagocytosis
Killing of phagocytosed bacteria
Peripheral findings of MDS
Cytopenias

PMNs -- hypogranular, segemental abnormalities

Erythrocytes -- macrocytes, ovalocytes, anisopolkicytosis, basophilic stipling, nucleated froms

Plts - large, hypogranular

Monocytes - monocytosis
May-Hegglin anomaly
has 1) dohle-like bodies 2) enlarged platelets and 3) thrombocytopenia
What color is the cytoplasm of a monocyte
gray-blue
lymphocytes are composed of ?
T and B cells
One of the gravest clinical problems for individuals with thalassemia major is:
Iron overload
Differential WBC count
means certain types of white cells elevated
More than 95% of an RBCs protein is this?
hemoglobin
Pathogenesis of Sickle Cell Anemia
HgS, upon deoxygenation, polymerizes with other HgS molecules in the same RBC, this causes distortion/ sickling of the RBC; Initially, this is reversible following re-oxygenation of the RBC, but after enough cycles it becomes irreversible
t(9;22) in ALL
Indicates a poor prognosis (it's a good prognostic factor in CML though)
Platelet Transfusion: Summary (Test question)
1. Patient with thrombocytopenia Evaluate severity of hemorrhage2. Determine etiology prior to platelet transfusion Harmful - TMA, PTP Helpful - Inadequate marrow production, DIC Nothing - ITP, PTP3. Check for refractoriness Pre and Post platelet counts
what physiologic deficiency results in B12 def.?
lack of intrinsic factor
how long does it take ferous sulfate to restore cbc's to norm
6 months
Rule of Three
In calculating the RBC indices the RBC count is usually 1/3rd of the Hemoglobin, and the Hemoglobin is usually 1/3rd of the Hematocrit. This gives a MCHC of 33.3 in a normal CBC.
What are the two types of aplastic/pancytopenic anemia?
rapid onset
slow onset
What blood cell?
Cell markers are CD3 & CD4
T helper cell
AML - M4
AMML 30 myeloblasts in marrow
M4e - w/eosinophilia
Describe acute lymphoid leukemia in PB.
-with/without blasts
-small blasts with scant blue cytoplasm and round nucleoli
-thrombocytopenia
Increased susceptibility to anaphylacic transfusion reaction
IgA deficiency
1 in 700
May have antibodies against all IgAs or a subtype that they are missing

Allergies to products that can be in blood (penicillin)
Hyperleukocytosis in ATRA treatment
Probably due to white cell maturation
Can cause leukostasis
Labs in fibrinolytic bleeding disorders
Prolonged PT and PTT
Increased D-dimers

(may look like DIC)
Folic Acid Deficiency Clinical manifestations are similar to those of cobalamin deficiency
Insidious onset
Absence of neurologic problems
Treated by replacement therapy
Encourage patient to eat foods with large amounts of folic acid
What is order from most to least of Leukocytes?
Neutrophils
Lymphocytes
Monocyte
Eosinophil
Basophil
Red cells that are resistant to malaria, rigid, and spoon shaped are termed:
Southeast Asian Ovalocytes
PRBC transfusion
10 ml/kg of RBCs raises crit 7-8%; should be freshly prepared and not more than 7 days old; should be of newborns ABO and Rh group and compatible with any ABO or atypical cell antibody present in the maternal serum
when less oxygen is delivered to the kidney a stimulus goes to the marrow and tells it to make more RBCs at a faster pace
what is hypoxia
Other than petechia and ecchymosis, what clinical manifestations are seen in DIC?
1)Bleeding from orifices 2) Acrocyanosis 3) PE 4) RF
Prognosis for Childhood ALL
Remission induced in 90% of childre with 2/3 of those effectively cured; Favorable Prognositc Factors - 2-10 years of age, Precursor B-cell type, hyperdiploidy, t(12;21) translocation; Unfavorable Prognostic Factors - translocations other than t(12;21), T-cell type,
HIV Definition
The presence of the HIV Virus in the individual's body
Blood donation tested for
"HIV, HBS/C, Hep C, p24, HTLV, Syphillis"
Autosomal disease causing an intrinsic defect in RBC membrane, due to loss of Ankyrin protein
Features of Hereditary Spherocytosis (a type of hemolytic anemia)
what drugs do you administer when you suspect an acute hemolytic reaction
epinephrine and vasopressors.
What are the symptoms of disseminated intravascular coagulation (DIC)?
bleeding from insertion sites
pallor
petechiae
oozing blood
hemoptysis
tachycardia
hypotension
bloody stools
hematuria
changes in mental status
pulmonary emboli
cyanosis of fingers and toes
cold agglutinin anemia etiology
IgM against RBC membrane in response to lymphoma, Waldenstrom, mycoplasma or mononucleosis, ulcerative colitis
RBCs destroyed in liver
What is the Diff Dx for eosinophilia?
NAACP
1. Neoplasm
2. Allergy/Asthma
3. Addison's disease
4. Collagen vascular disease
5. Parasites
Factors synthesized in liver
ALL

VIII is also made in endothelial cells
vWF is made only megakaryocytes and endothelial cells
Bad karyotypes in AML
Any 5 or 7 changes (including 5q)

Trisomy 8
Monsomy 7
What is the normal wbc in a canine?
6.0-17.0 X 10^3/microliter
how is folic acid deficiency tx?
with replacement therapy and diet
Transfusion related acute lung injury (TRALI)
pulmonary edema following transfusion; symptoms- dyspnea, cough, fever, hypo/hypertension
Desccribe RS cells.
Orig. from B cells, unique b/c only a minority of cell population, lg. size and binucleated
In Which part of the GI tract is B12 absorbed?
Terminal Ileum
LAP test distinguishes which 2 things
Leukemoid RXN (+) from CML (-)
What is a curative tx of sickle call anemia?
stem cell transplantation
What are the clinical manifestations of anemia?
- Response to hypoxia: dyspnea, rate/depth of breathing increases, palpitations, diaphoresis
- fatigue (even @ rest), dizziness, muscle pain b/c of decreased blood supply to cardiac/skeletal muscles
- pallor (pale lips, nail beds, and conjuctivae
- jaundice
- impaired healing/loss of elasticity
- increased cardiac output
- CHF
- edema
- paresthesias
- abdominal pain, nausea, vomiting, anorexia
- Low grade fever possible (b/c of pyrogens released by ischemic tissues)
Describe Hgb SC crystals.
Dark red color. One to two fingerlike projections; may look like a mitten. One to two per cell.
Characteristics of myeloproliferative disorders
Increase numbers in one or more cell lines, clonal marrow expansion

Extramedullary hematopoesis w/ hepatosplenomegally

Hypercatabolism (fevers, night sweats, weight loss)

Loss of sensitivity to growth factors

Predisposition to develop AML
What is a reticulocyte
equilibrium of dying rbcs and new rbcs being formed they can be aggregate or punctate
Cholelithiasis is a frequent complication of patients with hereditary spherocytosis because:
It causes the formation of gallstones and increased bilirubin due to hemolysis
Describe nodular sclerosing type of HL.
Fibrous bands separate the node into nodules and lacunar RS cells
What is another name for Factor 1?
Fibrinogen (Factor 1a - activated factor 1 - is known as fibrin)
Fetal RBCs made here at week 3
Embryonic visceral mesoderm gives rise to angioblasts intravascularly at this time
bone pain in leukemia is do to
the rapid production of abnormal cells
Why is PT elevated in DIC?
B/c of consumption of Factor VII, which has half-life of 4 hours
What do eosiniphil granules of a cow look like
small intense red jewel like
either a lower RBC count with a normal amount of hemoglobin in each cell
 
or a normal RBC number but now enough hemoglobin in the cells 
what two things cause anemia?
If a pt has no response to the initial tx for ITP, what is considered the definitive tx? What procedure is not indicated to tx ITP?
Splenectomy. Platelet transfusion.
What Are the Clotting Factors of the Common Pathway?
10, 2 (Prothrombin), 1 (Fibrinogen) and 1a (Fibrin)
Hb levels by age
At 2 days of age Newborn Hb level is ≥ 14.5gm/dL (mean 18.5 gm/dL)At 2 weeks of age Newborn Hb level is ≥ 12.5gm/dL (mean 16.5gm/dL)At 2 months of age Hb level is ≥ 9.0 gm/dL (mean 11.5gm/dL)At 2–12 years of age Hb level is ≥ 11.5gm/dL (mean 12.5gm/dL)At 12–18 y of age (males) Hb level is ≥ 13gm/dL (mean 14.5gm/dL).At 12 –18y of age (females) Hb level is ≥ 12gm/dL (mean 14.0gm/dL)
how is febril non hemolitic reaction caused
sensitive to wbc, platelets or plasma antigen.
Which RBC precursors are found in the blood normally? and with disease?
Nml - RBCs and reticulocytes

Disease -- all the way back to pro-normoblasts
The hemoglobin molecule consists of:
4 Heme molecules 4 Globin chains 2 Alpha 2 Beta Chains
what are the normal hemoglobin components in adults?
Hgb A1 (a2b2), A2(a2d2), and up to 2% F (a2f2)
G6PD deficiency leads to the formation of
Heinz Bodies by oxidation of Hgb in RBCs
what to do if you suspect febril nonhemolitic reaction
stop blood, keep ns going, give tylenol, monitor vs, call dr
when should iron be given and why?
should be givin an hour before meals b/c it does best in an acidic environment (thus vit c drink with it)
What is the confirmatory test in HIV testing?
Western Blot (do ELISA first, if it's positive, do a Western blot for confirmation)
When Do B and T cells become activated?
When they reach a specific antigen in the peripheral lymphoid organs otherwise are naive
What is the proximal cause of most RBC pathology in thalassemia?
RBC damage in thalassemia is caused by cell membrane damage (due to Heinz bodies and macrophages in the spleen)
-opathy
disease
MCV=
Hct/RBC count
thrombo
pertains to platelet
prorubricyte
12-17 μmN:C= 4:1
-phil(ic)
attracted to, affinity for
B cell with hypogammaglobulinemia
CLL
Phlebotomy Tubes
Phlebotomy Tubes
 
Red Tube
Chemistries
Amylase
ANA
CEA
Complement
Coombs
C-peptide
CPK isoenzymes
Cross-match
Folate
Glucose toleranse
Hepatitis
HIV
IgA, IgD, IgG, IgM
Iron, lipase, methemoglobin, most drug levels
PSA, RPR/VDRL, thyroid
 
Yellow Tube
Blood Cultures
 
Green Tube
Ammonia
Chromosome and HLA analysis
Thiamine
 
Blue Tube
PT, PTT, INR
Factor assays, fibrinogen, fibrin split products
 
Purple Tube
CBC, ABO type, cortisol, ESR
Helper T cell, renin (on ice)
 
Gray Tube
Ethanol
Lactate (on ice)
Sulfa levels
 
Poikilocytosis
Variation in RBC shape
Extravascular hemolysis

extrinsic causes
Liver disease
Hypersplenism
Inflections (malaria)
Oxidant agents (nitrites, dapsone)
Autoimmune hemolytic anemia
Intravenous immune globulin infusion
BASOPHILS
SMALL,RARE, INFLAMMATION AND ALLERGIC REACTIONS
antithrombin III inactivates
Thrombin and Xa
Megakaryocyte
The intermediate platelet precursor cell in the bone marrow, not normally present in peripheral blood. It is a large cell, usually having a multilobed nucleus that gives rise to blood platelets due to a pinching off of the cytoplasm.
DIC cont...
Clinical manifestations -mild ecchymosis, oozing to severe bleeding -hypoxia, cyanosis -neurological and renal impairment -microthrombi in sm blood vessels
calculating MCV
MCV= Hct X 10/RBC
Monocytes elevated in
Infection
Autoimmune, connective tissue disorders
Granulomatous disease (sarcoid, ulcerative colitis, etc)
FFP indications
replacement of coagulation factors
Prophy with abnormal coag studies
Treat microvascular bleeding with abnormal coag studies

THIS DOES NOT SEEM TO WORK
Normal range for Platelets
150-450 x10^9/L
What are Neutrophils often called??
Polymorphonuclear
not having enough hemoglobin
what is anemia?
Is conjugated bilirubin soluble or insoluble?
Soluble
Chemo with red urine (not blood)
Doxorubicin
Antiandrogens
Flutamide: Inhibits the uptake and/ornuclear binding of testosterone anddihydrotestosterone by prostatic tissue -most effective when combined with LHRH agonists. •Bicalutamide (Casodex) is a long lasting analog (once a day dosing) – also more selective for peripheral androgen receptor
Mast Cell
A tissue basophil. See Basophil.
pernicious anemia results because there is a lack in
intrinsic factorB-12
an abnormally small RBC is _________________
microcytic
Hgb
14-18 grams/dL Male

12 – 16 grams/dL Female
Symptoms of platelet hypofunction
epistaxis
gum bleeding
bruising
heave menses
petechiae
NOT hematomas or bleeding into joints
B12
Colbalomine
Cannot be produced by body
Animal products (meat, milk, eggs) only dietary source
Needs intrinsic factor to be absorbed
Treating DIC
Treat underlying cause

Support with plasma (factors), cryoprecipitate (fibrinogen), and plts RBCs

Heparin will stop, but with high risk of bleeding
Scott syndrome
Defect in platelet factor three

Bleeding disorder
Thrombocytopenia Nursing Management
Pain management
Hemarthrosis
Fluids & Electrolytes
Maintain hydration status (IV/PO) – blood loss leads to decreased circulating volume
What phagocytizes foreign material
Neutrophils and Macrophages
Lymphocyte concentrations in peripheral blood are greatest during what age interval?
Up to 4years
Hematoma
a collection of blood from internal bleeding that is sometimes clotted. It can be found in soft tissue or closed space in the body
normal PT
abnormal APTT
corrected by adsorbed plasma and serum
factor XI
factor XII
Pernicious Anemia
Autoimmune destruction of the gastric mucosa, which leads to a loss of parietal cells. Since the parietal cells are what make intrinsic factor, the levels of intrinsic factor are markedly decreased, which, in turn, means that B12 cannot be absorbed in the terminal ileum leading to a deficiency in B12.
AIDS Lymphoma
An important NHL Subtype; Aggressive lymphomas of B-cell origin; Burkitt's, Burkitt's-Like and Large Cell Immunoblastic Type; Treatment often limited by immune compromise of the patient; Prognosis improved with HAART therapy
Defective GPIb-IX-V platelet membrane receptor
Bernard Soulier syndrome
Carboplatin
• ovarian, non-small cell and small cell lung cancers• less toxic (~45×) than cisplatin but is generally less active
What type of control is erythropoesis under?
hormonally
what volume loss is it mostly noticable that the pt is in trouble
30
actquired (med use or geneticr/t exposure to myelotoxinspancocytopenia
aplastic anemia.
Iron deficiency anemia
Microcytic Hypochromic sm, pale RBC
What disorder is characterized by this cell?
May-Hegglin
This RBC form indicates?
Acanthocyte
Spiny appearance in abetalipoproteinemia
Reticulocyte

(not normal part of CBC)
0.5 – 1.5%
Evidence of dysmyelopoiesis may include any or all of the following:
-abnormal granulation
-abnormal nuclear shapes
-persistent basophilic cytoplasm
-uneven cytoplasmic staining
AML-- M2
AML with Maturation
30-45% of all AML

>10% pros, myelos, neutrophils in marrow

usually have granules and Auer rods
Bleeding phenotype of vWD
Ranges in severity
Mucocutaneous predominates
(mennorhagia, epistaxis, easy brusing, GI bleeding, oralpharyngeal)
Hemarthroses rare

Excessive bleeding with trauma, surgery

Long bleeding with minor cuts

Worse with aspirin
Systemic symptoms of HD
B symptoms
Pruitis
Alcohol induced pain
Agranulocytosis
failure bone marrow to make enough granulocytes (e.g. PMNs)
Hemophilia Nursing Management
Supportive care
Assess for symptoms; pain, bleeding
Bleeding precautions
Patient education
Genetic counseling
National hemophilia foundation
Medical alert bracelet
What is normal Hb in dog
12-18 g/dL
What feature is classic in the megaloblastic anemias:
Macro-ovalocytes
Hyperchromic & Hypochromic
increased MCH & decreased MCH
What causes relative polycythemia?
Dehydration which causes inc. RBC
Adult T-Cell Leukemia-Lymphoma
An important NHL Subtype; Associated with HTLV-I infection; Caribbean, Southeastern US; Hepatosplenomegaly, leukocytosis, lymphadenopathy, skin involvement, lytic lesions of bone, hypercalcemia; May respond to AZT and interferon
What is the normal range of an INR?
2-3
How long does scurvy take to develop?
4-8 months
Abciximab
Antibody to IIbIIIa receptor. used to prevent restenosis after an MI
What does too few RBC's lead to?
tissue hypoxia
Macrocyte
A red cell 9u in diameter or larger.
iron does what for the body
synthesis of hemoglobin
that test do you do with thrombocytopenia
tilt test
Myelodysplastic syndrome (MDS)
Also known as pre-leukemic syndrome Bone marrow suppression - low blood cell counts - WBC, RBC, platlets Abnormal blood cell production - production of immature lg myeloblasts Etiology -toxic exposures (benzene), chemo, radiation -Idiopathic Increase in older patients assoc w/CNL
What is the growth factor for T Cells?
IL-7
sideroblastic anemia
defect in Hb synthesis with trapped iron in mitochondria of RBCs
genetic --> ALA deficiency or defect in B6 metabolism
acquired --> chloramphenicol, isoniazid, alcohol, lead poisoning
can be associated with myelodysplastic syndromes and can progress to AML
initial tests --> ↑ferritin, ↑serum iron, ↓TIBC
confirmation --> marrow smear shows ringed sideroblasts in Prussian blue stain
treatment --> remove causing agent, lead and iron chelators, B6 in some cases
Describe aplastic anemia in PB.
Pancytopenia, normocytic normochromic (occasional macrocytes).
Mantle cell lymphoma pathogenesis
t(11:14) upregulates bcl-1 which increased cyclin D1 production
vWF deficiency Type 2
Normal levels
Reduced activity -- abnormal molecule or reduced number of high molecular weight multimers

20-30% of affected
Hemophilia Clinical manifestations
Persistent and prolonged bleeding from injuries
Delay of onset of bleeding
Subcutaneous echymosis and subcutaneous hematomas
Gingival bleeding
GI bleeding
Hematuria
Hemarthrosis
What is a hematophil?
Dark pink staining neutrophilic granules found in bird elephant and reptile
high blood viscosity
sickle cell anemia may develop cardipulmary disese
less than 20
Blast count in most individules is
Tx of AHTR?
1) Stop transfusion 2) Goal UOP >1mg/kg/hr 3) Analgesics 4) Pressors 5) Give blood prod. PRN
Laboratory Findings in Acute Leukemia
Leukopenia or Leukocytosis; Anemia; Thrombocytopenia; Circulating blasts (Myeloblasts in AML, Lymphoblasts in ALL)
Clinical Features of Beta Thalassemia
Profound growth retardation; Delayed sexual development; Gouty arthritis/ nephropathy; Cardiac malfunction, including congestive heart failure and fatal arrythmias, frequent causes of death; Cardiac dilation secondary to anemia is nearly universal - improves with transfusion, untreated leads to end-stage cardiomyopathy
CML
Dominant cell is neutrophil. Tip of 9 transferred to 22, philidelphia chromosome 210 KD protein (ALL 170 KD). Disease of adults,Peak age of incidence 53 years,Onset insidious,Presents in chronic phase,Splenomegaly related to WBC,1/3 patients have no symptoms at diagnosis.Imatinib (Gleevec),Dasatinib (Sprycel) are drugs of choice that inhibit the ATP binding domain.
What conditions of organs occur in sickle cell anemia?
hepatomegaly, jaundice, gallstones
what to give if pt has fluid v overload while giving blood
lasix
Common pathway of the coagulation cascade
Prothrombin activator splits prothrombin in the presence of Ca, produces thrombin Thrombin -ppolymerizes fibrinogen to long fibrin threads -activates enzyme fibrin stabilizing factor in plasma globulin -fibrin stabilizing factor caused covalent bonding and cross linkages to fibrin threads
What leukemia is this cell associated with?
Hairy Cell Leukemia
paroxysmal nocturnal hemoglobinuria etiology
red cell membrane defect in phosphatidyl inositol glycan A leads to increased complement lysis and intravascular hemolysis
What are significant causes of lymphocytosis?
Viral infection
Bacterial infection, esp pertussis
CLL
Leukemia on differential
Large increase in young forms -- acute

Increase in all forms with shift toward young - chronic
Major categories of normocytic anemias
Low reticulocyte -- hyproliferative with decreased production

High reticulocyte -- destruction of blood cells (reticulocyte)
What allows cells to differentiate and become "smart cells"?
fucntional non-condensed chromatin
What type of immunity is the inflammatory response?
Non-specific innate immunity
The monoclonal marker that is often positive in T-ALL is:
CD7 CD3
Anemia of premturity- symptoms
tachycardia, tachypnea, A's & B's, fatigue, poor weight gain, ^ O2 requirement, ^ lactate concentrations
Other than hypotension and fever common to transfusion reactions, what additional signs could suggest transfusion related acute lung injury (TRALI)?
Respiratory failure, bilateral pulm. edema
what differentiates a reticulocyte from a RBC?
reticulocytes still contain some RNA that remains for 1-2 days after the cell enters circulation, one that goes away it is a mature erythrocyte
Diagnosis of Hodgkin's Disease
Excisional Biopsy of a lymph node; Fine needle aspirate is NOT sufficient to make the diagnosis of Hodkin's Disease
What does DIC cause?
clotting AND bleeding at the same time, hard to treat
Differential Diagnosis for increased PTT in a patient with a bleeding disorder
Deficiency or inhibitor of Factors VIII, IX, or XI; vWD, heparin, lupus inhibitor with qualitative platelet defect
what are clinical manifestations of sickle-cell in children?
Short stature, delayed puberty, necrosis of metacarpals and metatarsals
3 basic functions of RBC membrane
1) separate intracellular from extracellular2) allow nutrient and ion passage selectively in and out of cell3) allow cell to deform when required
This RBC form indicates?
Target cell
HbC disease, Asplenia, Liver disease, Thalassemia
Describe iron deficiency anemia in PB.
Erythrocytes are hypochromic and microcytic; large variation in size; possible thrombocytosis.
Outcome of the coagulation cascade
Thrombin
Which convert fibrinogen to fibrin
Fibrin polymerizes around platlets and stabilizes
Creating the secondary hemostatic plug
Bone marrow biopsy in MDS
Hypercellular with at least one dysplastic lineage

Megakaryocytes dysplasia -- clustered cells, micromegakaryocytes, abnormal disorder

Erythroid -- delayed nuclear to cytoplasmic maturation, erythroid hyperplasia with megaloblastiod hyperplasia
-ringed sideroblasts can be seen
What is a hemogram
A complete detailed record of teh findings in a thorough examination of the blood, in reference to numbers, proportion and morphology
Spherocytes do not function well in the splenic environment because the:
Osmotic environment is impaired
How much can 1 dose of platelets raise the platelet count of the recipient?
30,000 to 60,000
Name 3 lab findings seen in a pt w/ ITP.
1) Plt
What is the Pathogenesis of Disseminated Intravascular Coagulopathy (DIC)?
Patients form clots everywhere in their body; They are consuming all of their clotting factors and fibrinolysis is increased as well - this is basically a vicious cycle - the patient is bleeding from everywhere (because of the increased fibrinolysis), but they're also in multi-system organ failure because their organs aren't getting enough blood due to the fact that the vessels supplying them have clotted off
Most Common Nitrosoureas and there properties
Carmustine (BCNU) and Lomustine (CCNU) Notable ADME: extremely high oil-to-water partition coefficient Used for glioblastoma Toxicity: DLT: bone marrow suppression, may persist due to storage of the drug in adipose and liver tissues Resistance: increased DNA repair Being replaced by: Temozolomide
Mean Corpuscular Volume (MCV)
A measure of the volume of red corpuscles expressed in cubic micrometers or femtoliters.
In which anemia are the symptoms fewer and milder than other anemias?
anemia of chronic inflammation
What are the diagnostic features of TTP (The Pentad)?
1. Microangiopathic hemolytic anemia (MAHA) - MUST BE PRESENT
2. Low platelets - MUST BE PRESENT
3. Fever
4. Neurological manifestations
5. Renal manifestations (hematuria, proteinuria, increased BUN/creatinine)
Hemophila A/B subtypes
80% A and 20 % B

Mild: 5-40% factor, bleed with trauma/surg, may have normal aPTT

Mod: 2-5% factor, may bleed spontaneous, prolonged aPTT

Severe: 0-1%, spontaneous bleeding common, prolonged aPTT
what is folic acid required for?
DNA sythesis leading to RBC formation
biliverdin than bilirubin than secreted into the intestines
when RBCs are broken down the heme part is broken down in what steps?
What dec. bleeding manifestations in vWD? Increases?
Pregnancy and estrogen. Inc. w/ age.
What is Adhesion (in Primary Hemostasis)?
Binidng of vWF to platelets - Specifically, it is the Glycoprotein Ib (GPIb) on the platelets binds to vWF; If a patient is lacking GPIb (a condition known as Bernard-Soulier) they are "tipped toward bleeding" since they can't bind platelets to vWF and can therefore not for a platelet plug
What is the result of Avitaminosis C?
decreased collagen formation --> weak vessels
What is the clinical presentation of hemolytic anemia?
toxicity of products of lysed RBCs
Abnormalities of RNC, desruction, or blood loss
Age related anemia Anemia of chronic disease (renal disease) Hemolytic anemia -short RBC life span -increased reticulocytes -acquired anemia: immune, traumatic, infective drugs, blood transfusion - incompatible break down RBC -hereditary - sickle cell, thalasemia
Do we treat CLL based on cytogenetics?
Not yet

Remember early treatment has not been shown to help
what anemia may be caused by adrenal hypo-function?
anemia of the chronic disase
Name 2 immune causes of platelet refractoriness?
1) Alloantibodies directed t/w class 1 HLA Ab 2) Autoantibodies in ITP
The exact etiology of TTP is unknown, but what are 3 factors associated to its dev.?
1) Estrogen 2) Pregnancy 3) HIV
Cause of the Decreased Haptoglobin in Hemolytic Anemia
Haptoglobin is a protein that binds free Hg (which is elevated in hemolytic anemia); Once it is bound to Hg, the is cleared by the liver - so with extra free Hg to be mopped up, extra haptoglobin will subsequently be cleared, decreasing its levels
Hyperhomocysteinemia is caused by low levels of these nutrients, and cause an increased risk of DVT and vascular disease
High blood levels of this are caused by folate (B9) and B12 deficiency
What is the difference between folate deficiency anemia and pernicious anemia?
In folate deficiency anemia, there is no neurological involvement.
What does the totoL Leukocyte count (WBC COUNT) Show us?
If there is leukocytosis or Leukopenia in wbc above or below normal ranges for that species
Why do granulocytes have to be crossmatched and Rh compatible? What is the expiration date of granulocytes?
Because they contain RBCs. 24hrs /p collection from donor.
Hemoglobin H Disease Form of Alpha Thalassemia
Deletion of 3 Alpha globin genes - (___, ___/ ___, Alpha); There's an excess of beta chains due to the underproduction of alpha chains, and these beta chains form tetramers, which are called Hemoglobin H; Moderately severe anemia; Dx - hemoglobin electrophoresis
Fetal RBC development during 2nd trimester
Fetal RBCs made in liver and spleen at this time
Describe Heinz bodies in New Methylene Blue stain.
Dark blue to purple in a mature erythrocyte. Composed of precipitated hgb. Single or multiple, generally membrane bound.
What is the other staging system used for lymphoma that divides pts into 3 groups?
Early favorable (stage I or II w/o risk factors), Early unfavorable (stage I or II w/ 1 or more RF), and Advanced (Stage III or IV )
What is alpha-thalassemia in which 3 genes are deficient called? what signs are present?
Hemoglobin H dz - typical facies and hepato-splenomegaly
A person w/ type O blood has what type of antigens? Ab?
Antigen: neither, Ab: anti-A, -B, and -AB
What is FFP? How often can it be donated?
Fresh frozen plasma, can be donated q 28d by aphoresis (must be frozen w/i 6hrs to maintain coagulation factors)
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