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A 35 year old man, non-alcoholic, an electrician by occupation and a resident of Delhi, was admitted with complaints of left upper abdominal discomfort, generalised weakness, lethargy, and exertional dyspnoea for the last five months. There was a history of two episodes of epistaxis five months previously. He did not have any history of weight loss, cough with expectoration, recurrent upper respiratory infections, haematemesis, melaena, or bleeding from any other site.
On examination, he was afebrile, markedly pale, anicteric, without any significant lymphadenopathy, petechiae, or sternal tenderness. His spleen was enlarged 12 cm below the left costal margin; it was not tender. The liver was also palpable 2 cm below the right costal margin. There was no ascites. His respiratory and cardiovascular examinations were normal.
Investigations revealed a haemoglobin concentration of 67 g/l, total leucocyte count of 1.3 × 109 cells/l (40% neutrophils and 60% lymphocytes), platelet count of 123 × 109/l, and erythrocyte sedimentation rate 60 mm in the first hour. His total serum protein levels were 86 g/l with reversal of albumin-globulin ratio (24 g/l and 62 g/l respectively). His renal profile, serum bilirubin, and aminotranferase levels was normal. Peripheral smear was negative for malarial parasite and did not show any abnormal cells. Abdominal ultrasound revealed massive splenomegaly (28 cm) and mild hepatomegaly with normal portal vein and splenic vein diameters. Echocardiography did not show any vegetations. IgG enzyme linked immunosorbent assay (ELISA) for HIV was negative. Blood levels of IgG, IgM, and IgA were also normal. At this stage a bone marrow examination was done (fig 1).
What does the bone marrow show?
What is the diagnosis and what further corroborative tests may be done?
What is atypical in this patient’s presentation?
How is this condition treated?