AZOTEMIA, HEMOLYTIC ANEMIA, WITH MINIMAL RENAL MICROANGIOPATHY IN A PATIENT WITH COMBINED IMMUNODEFICIENCY (CID), AND RHEOVIRUS (RV) INFECTION

Abstract

Hemolytic uremic syndrome (HUS) has been rarely reported in CID. A 3 month old male infant presented with 3 day history of vomiting, diarrhea, cough and 24 hr. oliguria. Two weeks prior to this episode patient was (pt) admitted and treated for diarrhea, dehydration, oligurea and azotemia. On second admission pt. had 10% dehydration, acidosis and oral thrush. A normal platelet count, coombs negative hemolytic anemia (Hct 33%, retic count 25%, burr cells), elevated BUN (62mg%), and creatinine (12mg%), metabolic acidosis (CO212mm/1, BE-15), microscopic hematuria and proteinuria were observed on admission. Subsequently the patient developed severe thrombocytopenia, moderate anemia and decreased GFR. These changes normalized in two weeks. A chest roentgenogram revealed pneumonitis and absence of thymus gland. Immunoglobulin electrophoresis and T-cell function were consistent with CID. Stool, CSF fluid and bone marrow cultures were positive for RV. oliguria, azotemia and hyponatremia responded to conservative therapy. However, pt. subsequently developed extensive dermatitis (D), generalized edema and recurrent bouts of diarrhea. Ten weeks later despite hyperalimentation and gammaglobulin therapy pt. expired. Autopsy revealed generalized RV infection, parainfluenza pneumonitis, hypoplasia of thymus, spleen and lymph nodes, graft vs. host D, and minimal glomerular histological changes. Whether mild renal histological changes seen in this pt. with other evidence of HUS is in part due to concomitant CID, is a matter of conjuncture.