“Unusual Cause Of Tophi With Renal Thrombotic Microangiopathy”

Abstract

Chronic neutrophilic leukemia (CNL) is a rare entity amongst myeloproliferative neoplasms (MPNs). The classical presentation of CNL is with splenomegaly, mature neutrophilic leucocytosis and hyperuricemia. We herein report a case who presented with symptoms of acute gouty arthritis. Physical examination showed typical red, tender tophi in the right hand, right foot and both pinnae suggesting an acute episode of gout. During evaluation, moderate splenomegaly, mature neutrophilia, hyperuricemia and subnephrotic range range proteinuria were noted. Bone marrow examination and kidney biopsy was done. Final diagnosis of CNL with acute gouty arthritis and chronic renal thrombotic microangiopathy (TMA) was made. Although hyperuricemia is a common finding in MPNs but presentation of our case with symptoms of acute tophi and chronic TMA is atypical. Case reportA-25-year-old gentleman with no previous comorbid illnesses presented to us in July 2010 with complaints of pain and swelling in multiple joints since the last 6 years. For past few years he used to have acute episodes of excruciating pain in the right great toe associated with redness of the overlying skin. General physical examination showed reddish, tender tophi in both pinnae, left 3rd metacarpal and right 1st metatarsal joints (Fig. 1a-c). X ray of the affected area showed erosion of the medial aspect of the right 1st metatarsal joint (Fig. 1d). Biochemical analysis showed elevated uric acid (15.6 mg/dL) with normal liver and renal function tests. Aspirate from left metacarpal swelling showed extensive deposition of needle shaped crystals confirming the diagnosis of gouty tophus (Fig. 2a, b). Abdominal examination was remarkable for splenomegaly 8 cm below the left costal margin. A complete blood count was performed that showed haemoglobin of 123 gm/L, platelet count of 320 9 10 9 /L and total white blood cell count of 26 9 10 9 /L with 83 % neutrophils, 10 % lymphocytes, 4 % basophils, 2 % eosinophils and 1 % monocytes on differential count. The neutrophils were morphologically normal and there were no immature cells in the peripheral blood. No evidence of chronic infections or occult malignancies that may be responsible for neutrophilia were identified on extensive investigation. A possibility of an MPN was considered in view of the symptom complex of hyperuricemia in addition to neutrophilic leukocytosis and splenomegaly. As depicted in the Table 1, our patient full filled all the criteria's required to make a diagnosis of chronic neutrophilic leukemia (CNL). Bone marrow examination was hypercellular with granulocytic and megakaryocytic hyperplasia without dysplasia and with a significantly high leucocytic alkaline phosphate score 318 (normal range: 40-100) (Fig. 3a-d). BCR-ABL transcripts and JAK2V617F mutation were negative by polymerase chain reaction.The acute episode of gout was managed with indomethacin and colchicine. Once the acute episode settled, he was started on allopurinol for hyperuricemia and hydroxyurea (1500 mg/day) as a cytoreducti...

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