Microangiopathic Hemolytic Anemia (MAHA) as Paraneoplastic Syndrome in Metastasized Signet Ring Cell Carcinomas: Case Reports and Review of the Literature

Zusammenfassung

Wir berichten über zwei Patienten mit spontan aufgetretener mikroangiopathischer hämolytischer Anämie (MAHA) bei metastasiertem Siegelringkarzinom des Magens und CUP (carcinoma of unknown primary). In beiden Fällen präsentierte sich die mikroangiopathische hämolytische Anämie als paraneoplastisches Syndrom. Klinisch fielen die Patienten durch eine akut einsetzende Coombs-negative, hämolytische Anämie mit Fragmentozyten im peripheren Blutausstrich auf, die typisch für das MAHA-Syndrom sind. Unter supportiver Therapie und einer eingeleiteten Chemotherapie nach dem ELF- bzw. nach dem PLF-Schema konnten wir den Tumorprogress und die Hämolyse kontrollieren.

Abstract

We report on two spontaneous cases of microangiopathic hemolytic anemia (MAHA) as first manifestation due to metastasized signet ring carcinoma, one of gastric and one of unknown origin. The patients presented with an acute onset of Coombs negative hemolytic anemia and fragmentocytes in the peripheral blood smear which are typical for MAHA. These case reports present MAHA as a rare paraneoplastic syndrome in patients with metastasized signet ring carcinoma. Parallel to symptomatic treatment we started chemotherapy treatment (ELF and PLF regimen, respectively). In both cases we were able to control the MAHA and cancer progression.

Schlüsselwörter

Magenkarzinom - Siegelringkarzinom - paraneoplastisches Syndrom - mikroangiopathische hämolytische Anämie

Key words

Gastric carcinoma - signet ring carcinoma - paraneoplastic syndrome - microangiopathic hemolytic anemia

References

• 1 Antman K H, Skarin A T, Mayor R J. et al . Microangiopathic hemolytic anemia and cancer: a review.  Medicine. 1979;  58 377-384
• 2 Ballas S K, Rubin R N. Case report: microangiopathic hemolytic anemia and thrombocytopenia with disseminated cancer.  Postgrad Med. 1976;  60 180-181
• 3 Nabeshima S, Kishihara Y, Nabeshima A. et al . Poorly differentiated adenocarcinoma with signet-ring cells of the Vater’s ampulla, without jaundice but with disseminated carcinomatosis.  Fukuoka Igaku Zasshi. 2003;  94 235-240
• 4 Schiller D. Malignancy-associated microangiopathic hemolytic anemia.  J Clin Oncol. 1997;  2474-2475
• 5 Clinicopathologic Conference . Microangiopathic anemia and disseminated intravascular coagulation in an elderly woman.  Am J Med. 1983;  74 1052-1060
• 6 Lynch E C, Bakken C L, Casey T H. et al . Microangiopathic hemolytic anemia in carcinoma of the stomach.  Gastroenterology. 1967;  52 88-93
• 7 Goodnight S H. Bleeding and intravascular clotting in malignancy: a review.  Ann NY Acad Sci. 1974;  230 271-288
• 8 Joseph R R, Day H J, Sherwin R M. et al . Microangiopathic hemolytic anemia associated with consumption coagulopathy in a patient with disseminated carcinoma.  Scand J Haematol. 1967;  4 271-282
• 9 Lohrmann H P, Adam W, Heymer B. et al . Microangiopathic hemolytic anemia in metastatic carcinoma: report of eight cases.  Ann Intern Med. 1973;  79 368-375
• 10 Marcos O O, Escuin E, Miguel J L. et al . Hemolytic syndrome in a patient with gastric adenocarcinoma: partial recovery of renal function after gastrectomy.  Clin Nephrol. 1985;  24 265-268
• 11 Linklater D M, Voth A. Thrombocytopenic purpura: importance of early diagnosis.  Can Fam Physician. 1996;  42 1985-1991
• 12 Murgo A J. Thrombotic microangiopathy in the cancer patient including those induced by chemotherapeutic agents.  Seminars in Haematology. 1987;  24 161-177
• 13 Tsuyoshi E, Hideo B, Akinobu T. et al . Diffuse bone metastasis with hematologic disorders from gastric cancer: clinicopathological features and prognosis.  Oncology reports. 1999;  6 601-605
• 14 Yoshikawa K, Kitaoka H. Bone metastasis of gastric cancer.  Jpn J Surg. 1983;  13 173-176
• 15 Miller S P, Davison T. Defibrination syndrome in cancer: treatment with heparin.  N Y State J Med. 1967;  67 452-458