Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Idiopathic Plasmacytic Lymphadenopathy with Polyclonal Hyperimmunoglobulinemia in a Patient who Died of Progressive Peripheral Polyneuritis and Cerebral Dysfunction
Kuniya KISHIMOTOTohru SAKATAKatsurou ITOHKazunori TOMINAGAHirohide INOIkuo MUROHASHIItsurou JINNAIMasami BESSHOHitoshi TAKEUCHIMasanobu SAITOHKunitake HIRASHIMA
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1997 Volume 38 Issue 2 Pages 117-123

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Abstract

We reported a 59-year-old woman who received a diagnosis of psoriasis vulgaris at the age of 35 and had been under medical treatment. She was admitted to our department on August 16, 1993 because of lymphadenopathy, arthralgia and neuralgia. We observed cervical and axillar lymphadenopathy 1∼3cm indiameter, anemia and leukothrombocytosis. Elevated levels of erythrocyte sedimention rate (ESR), C-reactive protein (CRP) and immunoglobulin G (IgG), but not M-protein were observed by immunological analysis of the serum. Bone marrow aspiration biopsy revealed hypercellularity with myeloid hyperplasia and slight increase in plasma cells. Elevated levels of serum interleukin-6 (IL-6) and granulocyte colony-stimulating factor (G-CSF) were detected; IL-6 was 62.1 pg/ml and G-CSF was 66 pg/ml, but IL-1α, IL-1β and TNF-α were within the normal range. Idiopathic plasmacytic lymphadenopathy (IPL) with polyclonal hyperimmunoglobulinemia was diagnosed by lymphnode biopsy and the patient received folluing treatment with prednisolone and hydroxyurea. Leukosytes, platelets and skin eruptions increased again when the steroid dose was tapered, so we changed treatments to MP (melphalan, prednisolone) therapy. In addition, various neurological abnormalities such as convulsions, loss of consciousness and peripheral polyneuritis were observed. Despite treatment her condition deteriorated and she finally died. Very few reports show these neurological abnormalities in IPL or Castleman's disease therefore we think this is a very rare case.

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© 1997 The Japanese Society of Hematology
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