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[Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly].
Rinsho Ketsueki. 2010 May; 51(5):320-5.RK

Abstract

We report three patients who presented with high fever, anasarca, hepatosplenomegaly, lymphadenopathy and severe thrombocytopenia accompanied by reticulin fibrosis of the bone marrow. This constellation of symptoms is not compatible with any known disease entity, and we had difficulty in diagnosis and treatment. A 47-year-old woman was suspected of having splenic lymphoma and received one course of CHOP regimen followed by continued steroid therapy. Her condition was improved but repeatedly became exacerbated with tapering of steroid. A 56-year-old man was treated with steroid pulse therapy and splenectomy without improvement. Histology of the liver and spleen did not show any specific findings. Immunosuppressive therapy with cyclosporin A was successful. Another 49-year-old man showed histological findings of paracortical hyperplasia with vascular proliferation and atrophic germinal centers on inguinal lymph node biopsy. These findings were similar to those of the hyaline-vascular type of Castleman disease or POEMS syndrome, but non-specific. Although he received steroid pulse therapy, he died of multiple organ failure. Autopsy demonstrated cytomegalovirus infection and hemophagocytic histiocytosis without malignant lymphoma. We suggest that this constellation represents a new clinical entity belonging to systemic inflammatory disorders with a background of immunological abnormality, requiring prompt and vigorous immunosuppressive therapy.

Authors+Show Affiliations

Division of Hematology, Niigata City General Hospital.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

jpn

PubMed ID

20534952

Citation

Takai, Kazue, et al. "[Thrombocytopenia With Mild Bone Marrow Fibrosis Accompanied By Fever, Pleural Effusion, Ascites and Hepatosplenomegaly]." [Rinsho Ketsueki] the Japanese Journal of Clinical Hematology, vol. 51, no. 5, 2010, pp. 320-5.
Takai K, Nikkuni K, Shibuya H, et al. [Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly]. Rinsho Ketsueki. 2010;51(5):320-5.
Takai, K., Nikkuni, K., Shibuya, H., & Hashidate, H. (2010). [Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly]. [Rinsho Ketsueki] the Japanese Journal of Clinical Hematology, 51(5), 320-5.
Takai K, et al. [Thrombocytopenia With Mild Bone Marrow Fibrosis Accompanied By Fever, Pleural Effusion, Ascites and Hepatosplenomegaly]. Rinsho Ketsueki. 2010;51(5):320-5. PubMed PMID: 20534952.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly]. AU - Takai,Kazue, AU - Nikkuni,Koji, AU - Shibuya,Hiroyuki, AU - Hashidate,Hideki, PY - 2010/6/11/entrez PY - 2010/6/11/pubmed PY - 2010/8/4/medline SP - 320 EP - 5 JF - [Rinsho ketsueki] The Japanese journal of clinical hematology JO - Rinsho Ketsueki VL - 51 IS - 5 N2 - We report three patients who presented with high fever, anasarca, hepatosplenomegaly, lymphadenopathy and severe thrombocytopenia accompanied by reticulin fibrosis of the bone marrow. This constellation of symptoms is not compatible with any known disease entity, and we had difficulty in diagnosis and treatment. A 47-year-old woman was suspected of having splenic lymphoma and received one course of CHOP regimen followed by continued steroid therapy. Her condition was improved but repeatedly became exacerbated with tapering of steroid. A 56-year-old man was treated with steroid pulse therapy and splenectomy without improvement. Histology of the liver and spleen did not show any specific findings. Immunosuppressive therapy with cyclosporin A was successful. Another 49-year-old man showed histological findings of paracortical hyperplasia with vascular proliferation and atrophic germinal centers on inguinal lymph node biopsy. These findings were similar to those of the hyaline-vascular type of Castleman disease or POEMS syndrome, but non-specific. Although he received steroid pulse therapy, he died of multiple organ failure. Autopsy demonstrated cytomegalovirus infection and hemophagocytic histiocytosis without malignant lymphoma. We suggest that this constellation represents a new clinical entity belonging to systemic inflammatory disorders with a background of immunological abnormality, requiring prompt and vigorous immunosuppressive therapy. SN - 0485-1439 UR - https://www.unboundmedicine.com/medline/citation/20534952/[Thrombocytopenia_with_mild_bone_marrow_fibrosis_accompanied_by_fever_pleural_effusion_ascites_and_hepatosplenomegaly]_ L2 - http://joi.jlc.jst.go.jp/JST.JSTAGE/rinketsu/51.320?lang=en&from=PubMed DB - PRIME DP - Unbound Medicine ER -