Goljan Pathology Synopsis Flashcards

Terms Definitions
HbSS osteomyelitis
Salmonella paratyphi
heparin neutralizing factor
Auer rods in myeloblasts
aplastic anemia; acute leukemia
Thymidylate synthetase
inhibited by 5-fluorouracil
intravascular hemolysis correlates with fever; falciparum-ring forms and gametocytes
synthesized in peritubular capillaries
fibrinogen and factor VIII
platelet receptor for fibrinogen
fever, thrombocytopenia, renal failure, hemolytic anemia with schistocytes, CNS deficits
inhibits sickling; hydroxyurea ↑ HbF
Aplastic anemia
drugs (e.g., phenylbutazone); infection (e.g., parvovirus); benzene
Micro-macroangiopathic hemolysis
mechanical damage causing intravascular hemolysis
Atypical lymphocytes
mononucleosis; CMV; toxoplasmosis; viral hepatitis; phenytoin
Polyclonal gammopathy
sign of chronic inflammation
Langerhan’s histiocytes
CD1 positive; Birbeck granules
↓ MCV, ↑ RBC count
tissue plasminogen activator, heparin, PGI2 ATIII, protein C/S
enhances ATIII activity (neutralizes all factors except V, VIII, fibrinogen)
Mast cells
release histamine (pruritus; swelling); metachromatic granules positive with toluidine blue
Gaucher’s disease
macrophages have fibrillary appearance; deficiency glucocerebrosidase
Antiphospholipid antibodies
lupus anticoagulant and anticardiolipin antibodies; vessel thrombosis
cleaves fibrinogen and insoluble fibrin into degradation products
activation coagulation system from release of tissue thromboplastin and/or endothelial cell damage
Circulating anticoagulants
antibodies destroy coagulation factors
Mature RBC
anaerobic glycolysis; no mitochondria or HLA antigens
t(9;22) of ABL POC; Philadelphia chromosome 22; ↓ alkaline phosphatase score
Nodal sites
germinal follicles, B cells; paracortex, T cells; sinuses, histiocytes
Clinical findings mono
exudative tonsillitis, generalized lymphadenopathy, hepatosplenomegaly
T cell deficiencies (HIV); combined B/T deficiency (adenine deaminase deficiency)
Chronic MPD
neoplastic stem cell disorder; splenomegaly; marrow fibrosis; risk for leukemia
Confirmatory tests
serum and urine immunoelectrophoresis; bone marrow aspirate
RBC size variation; ↑ iron deficiency; normal in other microcytic anemias
Hodgkin’s lymphoma
neoplastic component, Reed Stemberg (RS) cell; CD15 CD30 positive
AR; missense mutation (valine for glutamic acid 6th positive β-globin chain)
Causes sideroblastic anemia
alcohol, pyridoxine deficiency (isoniazid Rx of TB), Pb poisoning
Pathophysiology HbSS
vasoocclusive crises, hemolytic anemia (extravascular)
Pernicious anemia
autoimmune destruction parietal cells; chronic gastritis body/fundus; achlorhydria; ↑ gastrin
Vitamin B12
animal products; requires intrinsic factor for reabsorption in terminal ileum
Folate deficiency
MCC of increased serum homocysteine
B12 reabsorbed absorbed after administration of pancreatic extract
chronic pancreatitis
MCC of a prolonged bleeding time
Bleeding time
evaluates platelet function (adhesion, release reaction, aggregation)
Eosinophilic granuloma
benign histiocytosis; lytic bone lesions with pathologic fractures
Secondary amyloidosis
AA amyloid derived from serum-associated amyloid; chronic infections
MC infection transmitted by blood transfusion; MC antibody
positive direct Coomb’s test; spherocytes; MCC unconjugated hyperbilirubinemia first 24 hrs
% Saturation
↓ iron deficiency, anemia chronic disease; ↑ sideroblastic anemia
Serum ferritin
↓ iron deficiency; ↑ anemia chronic disease, sideroblastic anemia; normal thalassemia
Warm type AIHA
IgG; extravascular hemolysis; e.g., SLE, drugs
Leukemoid reaction
exaggerated WBC response to infection; usually due to infection
↓ in microcytic anemias; ↑ in spherocytosis
α-Thalassemia trait
AR; two α-globin gene deletions; normal Hb electrophoresis
Hb electrophoresis
HbAS-HbA 55-60%, HbS 40-45%; HbSS-HbS 90-95%, HbF 5-10%
Intestinal conjugase in folate metabolism
inhibited by phenytoin
Essential thrombocythemia
MPO with increase in abnormal appearing platelets
β-Thalassemia major
nonsense mutation with stop codon; hemolytic anemia; ↑↑ HbF, ↑ HbA2
Multiple myeloma
M spike; lytic bone lesions; pathologic fractures; hypercalcemia; renal failure
Congenital spherocytosis
AD; defect in spectrin; extravascular hemolysis; splenomegaly
ingredient in rat poison; danger to children in households with grandparents on warfarin
ATIII deficiency
no prolongation of PTT with administration of heparin
Duffy antigen
receptor for Plasmodium vivax; blacks often lack Duffy antigen
Febrile transfusion reaction
recipient anti-HLA antibodies react against donor leukocytes
Extravascular HTR
antibody attaches to donor RBCs; macrophage phagocytosis and hemolysis
Fresh frozen plasma
replacement for multiple factor deficiencies (e.g., cirrhosis, DIC)
Blood group A
increased incidence of gastric carcinoma
Blue fluorescent light
converts unconjugated bilirubin in skin into harmless water soluble dipyrrole
Alzheimer’s disease
amyloid precursor protein gene product chromosome 21; amyloid-β
Drugs and folate deficiency
alcohol, OC, phenytoin, methotrexate, trimethoprim, 5-fluorouracil
G6PD deficiency
XR; oxidant damage (peroxide) to Hb (e.g., primaquine; dapsone; fava beans)
S/S Pb poisoning adult
peripheral neuropathy; proximal renal tubule damage (Fanconi’s syndrome)
Blood findings in HbSS
sickle cells; target cells; Howell-Jolly bodies (nuclear remnants)
Monoclonal gammopathy
M component (spike); sign of plasma cell disorder
Relative polycythemia
↓ plasma volume; ↑ RBC count; normal RBC mass
Appropriate polycythemia
hypoxic stimulus for EPO to generate RBCs
Lab Pb poisoning
coarse basophilic stippling RBCs; ↓ MCV; ↑ blood Pb; ↑ d-aminolevulinic acid
Tests for vWF
ristocetin cofactor assay; vWF antigen assay; agar electrophoresis
Atypical antibodies
antibodies against Rh or non-Rh blood group antigens (e.g., anti-D)
S/S platelet dysfunction
cannot form temporary plug; epistaxis; petechiae; bleeding from scratches
Vitamin K deficiency
↓ epoxide reductase activity (↓ function vitamin K); hemorrhagic diathesis; ↑ PT
Polycythemia vera
↑ plasma volume and RBC mass; normal SaO2; ↓ EPO
Total iron binding capacity
↑ iron deficiency; ↓ anemia chronic disease, sideroblastic anemia
Mixed cellularity Hodgkin’s
male dominant; numerous RS cells; EBV association
Adult T cell leukemia
HTLV-1; CD4 T cells; skin infiltration; lytic bone lesions with hypercalcemia
Appropriate absolute polycythemia
normal plasma volume; ↑ RBC mass; ↓ SaO2; ↑ EPO
β-Thalassemia minor
AR; DNA splicing defect; ↑ HbA2 and F; ↓ HbA
Blood findings G6PD deficiency
Heinz bodies (denatured Hb; special stain); bite cells
von Willebrand factor
complexes with factor VIII to enhance VIII:C activity; platelet adhesion
Final common pathway factors
X, V, prothrombin (II), fibrinogen (I)
von Willebrand’s disease
AD; platelet adhesion defect + factor VIII deficiency
Stages iron deficiency
↓ ferritin; ↓ Fe and % saturation, ↑ TIBC; normocytic then microcytic anemia
Bence Jones protein
light chains in urine; predictive of a malignant plasma cell disorder
Protein C and S
inactivate factors V and VIII; enhance fibrinolysis
Ectopic EPO (renal cell carcinoma)
normal plasma volume; ↑ RBC mass; normal SaO2; ↑ EPO
Newborn physiologic anemia
drop in Hb due to replacement of HbF RBCs with HbA
Lab finding in circulating anticoagulant
prolonged PT and/or PTT corrected with mixing studies
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