Acute Myelogenous Leukemia M5b Developed during Clinical Remission of Castleman Disease


Castleman disease (CD) is a rare heterogeneous lymphoproliferative disease characterized by clinical symptoms due to an excess of interleukin-6 (IL-6) or IL-6-like activity. We describe the first case of CD associated with acute myelogenous leukemia (AML). A 55-year-old man presented with skin rash on his face and multiple cervical lymphadenopathy.The results of examination of his lymph node biopsy specimen led to a diagnosis of CD.The symptoms resolved after the administration of prednisolone.Three years after the onset of CD, the patient’s white blood cell count had increased to 63.4 x 109/L. His bone marrow aspirate showed that approximately 80% of cells were leukemic, including well-differentiated monocytic cells.A diagnosis of AML M5b was made. The patient died of invasive pulmonary aspergillosis after chemotherapy.

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  1. 1.

    Yoshizaki K, Matsuda T, Nishimoto N, et al. Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman’s disease.Blood. 1989; 74:1360–1367.

    PubMed  CAS  Google Scholar 

  2. 2.

    Soulier J, Grollet L, Oksenhendler E, et al. Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in multicentric Castleman’s disease.Blood. 1995;86:1276–1280.

    PubMed  CAS  Google Scholar 

  3. 3.

    Frizzera G, Peterson BA, Bayrd ED, Goldman A. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease: clinical findings and clinicopathologic correlations in 15 patients.J Clin Oncol. 1985;3:1202–1216.

    Article  CAS  PubMed  Google Scholar 

  4. 4.

    Molinie V, Perie G, Melo I, Melo C, Audouin J, Diebold J. Association of Castleman’s disease and Hodgkin’s disease. Eight cases and review of the literature.Ann Pathol. 1994;14:384–391.

    PubMed  CAS  Google Scholar 

  5. 5.

    Higashi K, Matsuki Y, Hidaka T, Aida S, Suzuki K, Nakamura H. Primary Sjogren’s syndrome associated with hyaline-vascular type of Castleman’s disease and autoimmune idiopathic thrombocytopenia.Scand J Rheumatol. 1997;26:482–484.

    Article  CAS  PubMed  Google Scholar 

  6. 6.

    Carrington PA, Anderson H, Harris M, Walsh SE, Houghton JB, Morgenstern GR. Autoimmune cytopenias in Castleman’s disease.Am J Clin Pathol. 1990;94:101–104.

    Article  CAS  PubMed  Google Scholar 

  7. 7.

    Marsh JH, Colbourn DS, Donovan V, Staszewski H. Systemic Castleman’s disease in association with Evan’s syndrome and vitiligo.Med Pediatr Oncol. 1990;18:169–172.

    Article  CAS  PubMed  Google Scholar 

  8. 8.

    Gerald W, Kostianovsky M, Rosai J. Development of vascular neoplasia in Castleman’s disease. Report of seven cases.Am J Surg Pathol. 1990;14:603–614.

    Article  CAS  PubMed  Google Scholar 

  9. 9.

    Chan JK, Fletcher CD, Nayler SJ, Cooper K. Follicular dendritic cell sarcoma. Clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized.Cancer. 1997;79:294–313.

    Article  CAS  PubMed  Google Scholar 

  10. 10.

    Bertero D, Buzio M, Albertino B, Giaccone M, Ricci E. A case report of Castleman’s disease in a patient with endometrioid adenocarcinoma of the ovary.Minerva Chirurg. 1991;46:989–993.

    CAS  Google Scholar 

  11. 11.

    Morishita H, Kobayashi T, Kunimi K, et al. A case of retroperitoneal Castleman’s disease associated with bladder tumor and a review of 59 cases in Japan.Acta Urol Jpn. 1992;38:1041–1044.

    CAS  Google Scholar 

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Correspondence to Akira Tomonari or Naoki Shirafuji or Arinobu Tojo or Tohru Iseki or Jun Ooi or Itaru Komiya or Kenzaburo Tani or Shigetaka Asano.

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Tomonari, A., Shirafuji, N., Tojo, A. et al. Acute Myelogenous Leukemia M5b Developed during Clinical Remission of Castleman Disease. Int J Hematol 77, 274–276 (2003).

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Key words

  • Castleman disease
  • Acute myelogenous leukemia
  • HHV-8
  • Autoimmune thrombocytopenia