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Unusual Sites of Hodgkin's Lymphoma

CASE 3. Cholemic Nephrosis in Hodgkin's Lymphoma With Liver Involvement

Charité University Medicine, Campus Benjamin Franklin, Berlin, Germany

A 40-year-old woman presented with fever, night sweats, and a progressive painless jaundice. Medical history included arterial hypertension, hyperlipidemia, and Sjögren's syndrome treated with azathioprine and prednisolone for the previous 6 months. No other drugs were taken.

On examination, liver and spleen were enlarged, but no peripheral lymph nodes were palpable. Abnormal laboratory tests included: hemoglobin 7.9 g/dL (normal range, 12 to 16 g/dL); WBC 3.01/nL (normal range, 4/nL to 10/nL); lymphocytes 3.57% (normal range, 25% to 40%); predominantly conjugated bilirubin 200 µmol/L (normal, < 22 µmol/L); alkaline phosphatase 1,145 U/L (normal, < 104U/L); -glutamyl-transferase 168 U/L (normal, < 38 U/L); AST 80 U/L (normal, < 35 U/L); creatinine 152 µmol/L (normal, < 80 µmol/L); urea 18.2 mmol/L (normal, < 8.3 mmol/L); total protein 53 g/L (normal range, 66 to 87 g/L); and albumin 21.5 g/L (normal range, 38 to 51 g/L). Coagulation times were within normal range. Urine analysis revealed urobilinogenuria (68 µmol/L), erythrocyturia (10/µL), and proteinuria (0.25 g/L). Serology for viral hepatitis type A, B, and C was negative. Extrahepatic obstructive cholestasis could be excluded by ultrasound imaging and endoscopic retrograde pancreaticocholangiography. Computed tomography scans of the thorax and abdomen revealed hepatomegaly otherwise inconspicuous, and splenomegaly as well as extensive mediastinal, abdominal, and retroperitoneal lymphadenopathy. A biopsy of the liver showed portal infiltrates of Hodgkin's lymphoma and Reed-Sternberg (HRS) cells in a background rich in eosinophils (Fig 1A). HRS cells were marked by a strong expression of CD30 (Fig 1B), and the Epstein-Barr virus–encoded latent membrane protein 1 (LMP1; not shown). Infiltration with HRS cells was also detected in bone marrow biopsy, which otherwise showed dysplastic features presumably related to preceeding azathioprine treatment. During the following few days, bilirubin had risen to 520 µmol/L, prothrombin time increased, anemia worsened, and thrombocytopenia developed. Renal function deteriorated; eventually, hyperkalemia developed, and hemodialysis was begun and carried out every other day. While ultrasound morphology of the kidney was normal, a fine-needle biopsy showed multiple greenish bile casts in the renal tubules, with degenerative changes of the tubular cells but no evidence of lymphoma cells (Fig 2), yielding the diagnosis of acute renal failure due to cholemic nephrosis. After chemotherapy with dose-reduced doxorubicin, bleomycin, vinblastine, dacarbazine (ABVD) regimen, cholestatic parameters and liver function rapidly improved along with renal function restoration, so that hemodialysis could be discontinued.

View larger version (72K): [in this window] [in a new window]   Fig 1.

View larger version (132K): [in this window] [in a new window]   Fig 2.

Authors' Disclosures of Potential Conflicts of Interest
The authors indicated no potential conflicts of interest.

REFERENCES

1. Cervantes F, Briones J, Bruguera M, et al: Hodgkin's disease presenting as a cholestatic febrile illness: Incidence and main characteristics in a series of 421 patients. Ann Hematol 72:357-360, 1996[Medline]

2. Dabbs DJ, Morel-Maroger Striker L, Mignon F, et al: Glomerular lesions in lymphomas and leukemias. Am J Med 80:63-70, 1986[CrossRef][Medline]

3. Bairaktari E, Liamis G, Tsolas O, et al: Partially reversible renal tubular damage in patients with obstructive jaundice. Hepatology 33:1365-1369, 2001[CrossRef][Medline]

4. Bomzon A, Holt S, Moore K: Bile acids, oxidative stress, and renal function in biliary obstruction. Semin Nephrol 17:549-562, 1997[Medline]

5. Holmes TW Jr: The histologic lesion of cholemic nephrosis. J Urol 70:677-685, 1953[Medline]

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This Article Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Kiewe, P. Articles by Thiel, E. Search for Related Content PubMed PubMed Citation Articles by Kiewe, P. Articles by Thiel, E. Social Bookmarking                            What's this?