Hemolytic uremic syndrome associated with Plasmodium vivax malaria successfully treated with plasma exchange
A 29 year old previously asymptomatic male was admitted in a peripheral hospital with fever and jaundice of 1 week duration. He had anemia (hemoglobin [Hb] 8.8 g/dl), thromocytopenia (platelet count 55,000/μl), renal impairment (serum creatinine [s. creatinine] 1.5 mg/dl) and direct hyperbilirubinemia (total bilirubin 3.9 mg/dl, direct 1.9 mg/dl). Peripheral smear showed the presence of schizonts of Plasmodium vivax. Antigen test for Plasmodium falciparum was negative. He was treated with Artesunate and Clindamycin for 7 days and was discharged when asymptomatic, although improvement in thrombocytopenia or renal impairment was not observed. Two week later (1 week after being afebrile and getting discharged) he presented to us with vomiting and pain in the abdomen for 3 days and decreased urine output. He did not have fever, loose motions, rash, bleeding or alteration in sensorium. There was no history of treatment with quinine. He was hypertensive, pale, did not have edema and had unremarkable systemic examination except for the presence of haemic murmur. Severe anemia (hemoglobin 4.8 g/dl), thrombocytopenia (platelet count 61,000/μl) and azotemia (s. creatinine 8.3 mg/dl) were noted. Presence of schistocytes on peripheral smear, elevated reticulocyte count (13%), elevated serum lactate dehydrogenase (LDH 1242 U/L) and indirect hyperbilirubinaemia indicated ongoing microangiopathic hemolytic anemia. Urinalysis showed microhematuria and proteinuria of 3.75 g/day. Coagulation profile was normal. Serum C3 level was low (50 mg/dl), C4 level being normal. Antinuclear antibodies and enzyme-linked immuno sorbent assay for human immunodeficiency virus were negative. Hepatitis B surface antigen and anti-hepatitis C antibodies were negative. He underwent five sessions of 1.5 volume plasma exchanges with replacement with fresh frozen plasma. Hypertension was controlled. Intermittent hemodialysis was done. Anemia was corrected with packed red cells transfusion. After five sessions of plasma exchanges, platelet count increased to 150,000/μl Hb stabilized at 6 g/dl and then increased. S. LDH decreased to 206.3 U/L and s. creatinine stabilized at 5.5 mg/dl, patient being dialysis-independent. This hematological remission persisted at 4th week (Hb 9.1 g/dl, platelet count 163,000/μl) with C3 level being normal. Kidney biopsy was performed at this time. Enlarged glomeruli had capillary wall thickening, with double contours seen on silver Methanamine stain, endothelial swelling and increased mesangial cellularity. Arterioles with onion peel appearance were seen [Figures and ]. No immune deposit was seen on immunoflorescence study.\nAt the end of 4 months, he requires two antihypertensive drugs, has Hb 13.5 g/dl, platelet count 229,000/μl, s. creatinine 1.4 mg/dl and poteinuria 1.2 g/day.