A febrile patient with polyarthritis, visceromegaly and cytopenias
Keywords:Arthritis, Hepatomegaly, Splenomegaly, Cytopenias
An unusual case of febrile patient i.e., polyarthritis which was asymmetrical with hepatosplenomegaly, joint pain for last 6 months, liver size of 9cm and spleen 6cm. Lab investigations revealed decreased all blood lines including TLC 1830μl, Hb 9.5g/dl, platelets 44000mL, HCT 27.3% and MCV 69 L/cell, with raised erythrocyte sedimentation rate (ESR). Previous studies for rheumatoid arthritis (RA) factor were negative. Patient came to Akbar Niazi Teaching Hospital was evaluated for pancytopenia, collagen vascular disorder, RA and SLE (probable hematological pathology as patient of SLE or some other secondary diagnosis).
Bone marrow biopsy (BMB) was done which revealed hairy cell leukemia. Further confirmation is done by immune-histochemical (IHC); meanwhile antinuclear antibody (ANA) was positive, workup revealed Anti-dsDNA antibodies, patient in painful arthritis and started on steroids.
As the IHC was awaited, patient was discharged home for two week on steroids. After confirmation of hairy cell leukemia on IHC, patient was recalled for further management plan. Surprisingly the blood CBC report was showed; initial TLC 1830μl, Hb 9.5g/dl, platelets 44000mL, HCT 27.3% and MCV 69 L/cell. Last CBC was showed, TLC 5500μl, HB 12 g/dl and platelets 16000 ml.
Now the distinguishing point is, unlikely for hairy cell leukemia to respond steroids so quickly within 10 days, or this was all SLE related manifestations and HCL was an incidental finding during the workup of pancytopenia. On further followup we will do BRAF and flow cytometry for HCL.
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