Summary
A patient is described with a rapidly progressive Kaposi's sarcoma following immunosuppressive therapy with fludarabine monophosphate (FAMP) for chronic lymphocytic leukemia. The development of this tumor was accompanied by progressive thrombocytopenia, due to trapping of the platelets in the Kaposi sarcoma, without signs of consumptive coagulopathy or microangiopathic hemolytic anemia. This sequestration process might be caused by the abnormal structure of the tumoral vessels, leading to exposure of subendothelial structures like collagen, von Willebrand's factor, and tenascin to the vessel lumen. A severe immunosuppression due to the lymphocytotoxic effect of FAMP on several T-cell subpopulations was seen. This case confirms the importance of immunosuppression as a cofactor in the development of Kaposi's sarcoma also in HIV-negative patients, and adds Kaposi's sarcoma to the differential diagnosis of thrombocytopenia in immunosuppressed patients.
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References
Kaposi M (1872) Idiopathisches multiples Pigmentsarkom der Haut. Arch Dermatol Syphilol 4:265–273
Piette WW (1987) The incidence of second malignancies in subsets of Kaposi's sarcoma. J Am Acad Dermatol 16:855–861
Penn I (1979) Kaposi's sarcoma in organ transplant recipients. Transplantation 27:8–11
Anonymous (1981) Kaposi's sarcoma andPneumocystis pneumonia among homosexual men. New York City and California. MMWR 30:305–307
Gange RW, Wilson-Jones E (1978) Kaposi's sarcoma and immunosuppressive therapy: an appraisal. Clin Exp Dermatol 3:135–146
Safai B, Mike V, Giraldo G, Beth E, Good RA (1980) Association of Kaposi's sarcoma with second primary malignancies. Possible aetiopathogenic implications. Cancer 45:1472–1479
Moertal CG, Hagedoorn AB (1957) Leukemia or lymphoma and coexistent primary neoplasms: a review and a study of 120 cases. Blood 12:788–311
Wijermans PW, Gerrits WBJ, Haak HL (1993) Severe immunodeficiency in patients treated with fludarabine monophosphate. Eur J Haematol 50:292–296
Bergmann L, Fenchel K, Jahn B, Mitrou PS, Hoelzer D (1993) Immunosuppressive effects and clinical response of fludarabine in refractory chronic lymphocytic leukemia. Ann Oncol 4:371–375
Friedman-Birnbaum R, Weltfriend S, Pollack S (1991) Classic Kaposi's sarcoma: T-lymphocyte subsets, T4/T8 subsets and NK cell activity. J Am Acad Dermatol 24:937–940
Harwood AR, Osoba D, Hofstader SL (1979) Kaposi's sarcoma in recipients of renal transplants. Am J Med 67:759–765
Peterman TA, Jaffe HW, Friedman-Kien AE, Weiss RA (1991) The aetiology of Kaposi's sarcoma. Cancer Surv 10:23–37
Zukerberg LR, Nickoloff BJ, Weiss SW (1993) Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasma associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol 17:321–328
Enzinger FM, Weiss SW (1988) Soft tissue tumors, 2nd edn. Mosby, St. Louis
Poggi A, Stella M, Donati MB (1993) The importance of blood cell-vessel wall interactions in tumour metastasis. Baillieres Clinical Haematol 6:731–752
Kosfeld MD, Frazier WA (1993) Identification of a new cell adhesion motif in two homologous peptides from the COOH-terminal cell binding domain of human thrombospondin. J Biol Chem 25:8808–8814
Reynolds WA, Winkelman RK, Soule EH (1965) Kaposi's sarcoma: a clinicopathologic study with particular reference to its relationship to the reticuloendothelial system. Medicine 44:419–443
Ih-Jen S, Tseng-Tong K, Shou-yien W, Iou-Jih H (1984) Lymphadenopathic type of Kaposi's sarcoma presenting with generalized petechial hemorrhages. A case report from Taiwan. Cancer 54:948–950
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Wijermans, P.W., van Groningen, K., van Royen, E.A. et al. Kaposi's sarcoma in an HIV-negative CLL patient as the cause of thrombocytopenia. Ann Hematol 68, 307–310 (1994). https://doi.org/10.1007/BF01695038
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DOI: https://doi.org/10.1007/BF01695038