İrfan SOYKAN, Ramazan İDİLMAN, Meral BEKSAÇ, Mutlu ARAT, Sibel ERTEK, Cemil EKİNCİ

Fatal fulminant hepatic failure during treatment of multiple myeloma

Karaciğer, hematolojik hastalıklarda en sık tutulan organlardan birisidir. Multipl myeloma ekstramedüller ve kemik dışı tutulumu nadir görülen bir hematolojik hastalıktır. Karaciğer tutulumu ile ilgili çalışmalar az da olsa, bazı postmortem çalışmalar hepatik plazma hücre infiltrasyonunu gösterebilmiştir.Bu yazıda 58 yaşında, üç yıl önce multipl myelom tanısı alan, 5 kür VAD kemoterapi protokolü, talidomid ve iki kez paravertebral tutulum bölgelerine radyoterapi uygulanmış bir erkek hastayı sunuyoruz. Hastada terapötik plazma değişimlerine ve destek tedaviye rağmen karaciğer yetmezliği ilerlemiş ve hasta fulminan karaciğer yetmezliği nedeniyle kaybedilmiştir. Postmortem karaciğer biyopsisinde myelomatöz tutulumla ilişkili bulgu gösterilememiştir. Bu yazıda hastalığın fulminan seyrini ve post-mortem karaciğer biyopsisinde sadece non-spesifik reaktif hepatit bulunan multipl myelom hastasında karaciğer yetmezliğinin nedenlerini değerlendirdik.

Multipl myeloma tedavisi sırasında ölümcül fulminan karaciğer yetmezliği

Liver is one of the most commonly involved organs in hematological diseases. Multiple myeloma (MM) is a hematological malignancy with rare extraosseous and extramedullary involvement Although there is a few clinical data about hepatic involvement postmortem evaluations were able to demonstrate plasma cell infiltration in liver. We are reporting a 58-year-old male patient who had the diagnosis of MM since three years and had received five courses of VAD chemotherapy, thalidomide and two courses of radiotherapy to paravertebral involvement sites. The patient progressed and received VAD courses as salvage chemotherapy. He developed hepatic failure and despite consequent therapeutic plasma exchanges and supportive measures the patient status worsened and he died of fulminant hepatic failure. We were not able to demonstrate any sign of myelomatous involvement in post-mortem liver biopsy. We are reporting the fulminant disease course and discussing the possibilities for hepatic failure in a MM patient, whose postmortem liver biopsy revealed only non-specific reactive hepatitis.

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1. Lee RG, Bithell TC, Foerster J, et al. Wintrobe’s Clinical Hematology, ed 9, Vol.2; 2225.
2. Walz-Matmüller R, Horny HP, Ruck P, et al. Incidence and pattern of liver involvement in haematological malignancies. Pathol Res Pract 1998; 194:781-789.
3. Perez-Soler R, Esteban R, Allende E, et al. Liver involvement in Multiple myeloma. Am J Hematol 1985; 20:25-29.
4. Morgan D, Cieplinski W. Case report: Myelomatous ascites. Am J Med Sci 1985; 290:159-164.
5. Braunwald E , Fauci AS, Kasper DL, et al. Harrison’s Principles of Internal Medicine, ed 15, vol 2 ,1739.
6. Peuckmann V, Fisch M, Bruera E. Potential novel uses of thalidomide. Drugs 2000; 60:273-292.
7. Singhall S and Mehta J; Thalidomide in cancer. BioDrugs 2001; 15:11-10.
8. Mornex F, Gerard F, Ramuz O, et al. Late effects of radiations on the liver. Cancer Radiother 1997; 1:753-759.
9. Cromheecke M, Konings AW, Szabo BG, et al. Liver tissue tolerance for irradiation: experimental and clinical investigation . Hepato-Gastroenterol 2000; 47:1732-1740.
10. Araki K, Yamamoto H, Fujimoto J. Studies on the pathogenesis of murine experimental autoimmune active hepatitis: sensitized T cell involvement in its induction. Clin Exp Immunol 1987; 67:326-334.
11. Moake LM. Mechanisms of disease: Thrombotic microangiopathies. New Engl J Med 2002; 347:589-600.