Ascites in the “TAFRO” Syndrome: Does the Squeeze Make the Juice?

Excerpt

A 41-year-old healthy female underwent cholecystectomy due to symptomatic gallstone related acute cholecystitis. A week later she was readmitted with fatigue, low grade fever, worsening pallor, generalized edema including bilateral leg swelling and abdominal distension with pain. Laboratory evaluation showed mild normocytic anemia (Hgb 7 g/dL [12–16 g/dL]), mean corpuscular volume 92 fL (80–100 fL), mild leukocytosis with WBC count 12,000/µL (4000–11,000/µL), thrombocytopenia (platelet count 55,000/µL [150,000–400,000/µL]). Comprehensive metabolic panel showed normal serum electrolytes, blood urea nitrogen, creatinine, and liver profile, except for low serum total protein of 5.1 g/dL (6.1–8.2 g/dL) and hypoalbuminemia (albumin 2.4 g/dL [3.4–4.7 g/dL]). Urinalysis showed proteinuria and an elevated random protein-to-creatinine ratio of 3.8. A 24-h urine collection revealed an elevated total protein of 3.1 gm/24 h, consistent with nephrotic range proteinuria. A contrast computerized scan (CT) of chest, abdomen and pelvis showed moderate bilateral pleural effusions, and moderate ascites: no other abnormality was noted in the chest, abdomen, or pelvis. Diagnostic and therapeutic paracenteses were performed draining about 3000 ml of straw-colored fluid. Analysis showed a serum-ascites-albumin gradient (SAAG) of 1.9 g/dL with a normal cell count and negative cytology. An extensive serologic and autoimmune investigation was undertaken for anemia, hypoalbuminemia, and thrombocytopenia. Anti-nuclear antibody (ANA) was mildly elevated at 1:160 titer (homogenous pattern). Anti-Smith, anti-Jo1, anti-smooth muscle antigen (ASMA), antimitochondrial antibody (AMA), anti–liver-kidney microsomal antibody (LKM), anti-neutrophilic cytoplasmic antibody (ANCA), aldolase, anti-synthetase, anti-ribonuclear protein, anti-dsDNA, anti-myeloperoxidase, anti-dermatomyositis antibody panel were negative. Infectious workup including syphilis, human immunodeficiency virus, EBV, HSV, CMV, parvovirus B19, and antistreptolysin O (ASO) titer are unremarkable. Hematologic tests for haptoglobin, ADAMTS 13, cryoglobulins, serum and urine protein electrophoresis and lactate dehydrogenase were all negative. A CT of abdomen and pelvis (without contrast) now showed a large volume of ascites, moderate bilateral pleural effusions and, for the first time, some mildly prominent retroperitoneal, pelvic, and inguinal lymph nodes. Kidney biopsy revealed a global, diffuse thrombotic micro-angiopathy; the bone marrow, though hypercellular for age, displayed no dysplastic changes, no increase in blast cells: no monoclonal B-cells and abnormal T-cells were absent. Based on these findings, a diagnosis of thrombotic thrombocytopenic purpura (TTP) was made, and therapy was started with high doses of intravenous dexamethasone and repeated plasma exchanges. The patient, however, did not respond, and after a multi-disciplinary meeting between rheumatology, hematology, and nephrology services, the diagnosis was revised to atypical/complement mediated hemolytic uremic syndrome (HUS) that was treated with eculizumab. Blood platelet and hemoglobin concentrations improved, and renal dysfunction and proteinuria gradually resolved. The ascites, however, persisted, which necessitated diuretic therapy with furosemide and spironolactone. …