[Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly].
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.
Takai
Kazue
K
Division of Hematology, Niigata City General Hospital.
Nikkuni
Koji
K
Shibuya
Hiroyuki
H
Hashidate
Hideki
H
D001201:Ascites / Q000209:etiology*; D016572:Cyclosporine / Q000008:administration & dosage; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D005334:Fever / Q000209:etiology*; D006529:Hepatomegaly / Q000209:etiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D010996:Pleural Effusion / Q000209:etiology*; D011239:Prednisolone / Q000008:administration & dosage; D055728:Primary Myelofibrosis / Q000209:etiology*; D013163:Splenomegaly / Q000209:etiology*; D013577:Syndrome; D013921:Thrombocytopenia / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011239:Prednisolone