608-2883-1-PB.pdf - International Journal of Advances in Medicine Chetiwal R et al Int J Adv Med 2017 Aug;4(4:1189-1192 http/www.ijmedicine.com pISSN

608-2883-1-PB.pdf - International Journal of Advances in...

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International Journal of Advances in Medicine | July-August 2017 | Vol 4 | Issue 4 Page 1189 International Journal of Advances in Medicine Chetiwal R et al. Int J Adv Med. 2017 Aug;4(4):1189-1192 pISSN 2349-3925 | eISSN 2349-3933 Case Report Anemia with massive splenomegaly an uncommon presentation of osteopetrosis in adult Rajesh Chetiwal 1 *, Ajit K. Naik 1 , O. P. Lakhwani 2 INTRODUCTION Anemia with massive splenomegaly is not an uncommon clinical condition. The patients of Hematological disorders like hemolytic anemia, chronic myeloid leukemia, myelofibosis and other myeloproliferative disorders. In parasitic infections like malaria and kala- azar, anemia with massive splenomegaly is not an unusual feature. Patients of Osteopetrosis presenting as anemia and massive splenomegaly in adult is an uncommon presentation. Osteopetrosis is characterized by excessive density of bones leading to similar radiological appearance of cortical and medullary compartments of bones, is a hallmark of "chalk bone” appearance on skeletal x-ray. There is a defect in osteoclastic activity which leads to failure in remodeling of developing bone. Obliteration of marrow cavities leads to development of secondary anemia (myelophthisic anemia). 1-8 . Type A, autosomal recessive, commonly seen in children is sever form of disease and death before puberty. 4 Type B of osteopetrosis is an autosomal dominant also known as adult type, it is less severe and usually possess a benign disease course and most of the time diagnosed incidentally in adult. 1 CASE REPORT A 31-year-old female, house wife, resident of Etawah Uttar Pradesh, presented with history of easy fatigability of long duration 10 years, abdominal discomfort in the ABSTRACT Osteopetrosis was first described by a Germen radiologist Albers - Schoenberg in 1904. It is characterized by excessive density of bones leading to typical "chalk bone" appearance on skiagram. There is a defect in the osteoclastic activity which leads to failure in remodeling of the developing bone. This excessive formation of bone with defective osteoclastic activity leads to mechanically weak bone. So, fractures on minor trauma are common presentation of the disease. Obliteration of marrow cavities leads to development of secondary anemia which is a feature of infantile form of osteopetrosis (malignant osteopetrosis), usually seen in infants and early childhood. Adult form of osteopetrosis known as benign osteopetrosis may remain asymptomatic and causes delay in the early diagnosis of it. Anemia and massive splenomegaly in adult is a very uncommon presentation of the osteopetrosis, as seen in this interesting case which we are discussing further in detail. Keywords : Myelophthisic anemia, Marble bone, Osteopetrosis, Skeletal dysplasia, Splenomegaly 1 Department of Medicine, ESI, PGIMSR, Basaidarapur, New Delhi - 110015, India 2 Department of Orthopaedics, ESI, PGIMSR, Basaidarapur, New Delhi - 110015, India Received: 10 April 2017 Revised: 13 June 2017 Accepted: 17 June 2017 *Correspondence: Dr Rajesh Chetiwal, E-mail: [email protected]
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