Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0151825 (bone pain)
 3,118 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of anaplastic large cell Ki-1 lymphoma involving bone as the most prominent and initial manifestation are reported. The first patient was a 20-year-old male who had back pain and incomplete paraparesis due to vertebral involvement. The second was a 14-year-old girl, whose first clinical signs were fever of unknown origin and sternal bone pain. Radiologically, skeletal lesions were lytic and destructive. Histopathologically, the tumour cells had pleomorphic bizarre nuclei and abundant basophilic cytoplasm. Immunohistochemically, Ki-1(CD30) reactivity was strongly positive in both cases. Tumour cells were also CD3, CD4, epithelial membrane antigen and interleukin-2 receptor positive in the first case, and CD10, HLA-DR positive in the second case. The former tumour was considered to be of T-cell lineage and the latter of lymphoid progenitor cell origin. Radiation and chemotherapy were temporarily effective. However, both patients died 14 and 7 months after diagnosis, respectively, due to systemic lymph node involvement. These observations suggest that the prognosis for Ki-1 lymphoma involving bone is poorer than indicated in previous reports.
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PMID:Anaplastic large cell Ki-1 lymphoma with bone involvement: report of two cases. 755 39

Non-Hodgkin's lymphomas uncommonly present as bone lesions. Most of these tumors are diffuse large B-cell lymphomas. Anaplastic large cell lymphoma (ALCL) presented as bone lesions is exceedingly rare. In this study, we describe six cases of ALCL that presented as solitary or multiple bone lesions. The average patient age was 33 years (range, 4 to 63 years) and the male to female ratio was 2:1. Fever and localized bone pain were the most frequent presenting symptoms. Radiologic examinations revealed osteolytic lesions in all cases, with three (50%) being multiple lesions and five (83%) involving the axial bones. All patients were initially assessed to have only bone involvement. Staging studies revealed mild cervical lymphadenopathy in one patient and no evidence of extraskeletal disease in the other five patients. Histologically, there was diffuse infiltration of one or more bones by large pleomorphic lymphoma cells. Immunohistochemical studies showed all six neoplasms were positive for CD30, EMA, and granzyme B. One case was of T-cell lineage, positive for CD3. One case was positive for the T-cell-associated antigen CD4. The remaining four cases were of null-cell type. In-situ hybridization for EBV was performed in five cases; all were negative. Despite the relatively low International Prognostic Index (IPI) of these patients (mean, 1.67; range, 1 to 3), the overall prognosis was relatively poor: three of six died of disease within 2 years of diagnosis, and two of six were alive with evidence of disease (follow-up, 6 mo to 2 years). Thus, compared to their nodal counterparts, ALCLs that present as bone lesions are distinguished by their uniform expression of EMA and granzyme B, and a relatively poor clinical outcome. Our results also suggest that ALK-1 expression in this clinical setting is not a favorable prognostic indicator.
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PMID:Anaplastic large cell lymphomas presented as bone lesions: a clinicopathologic study of six cases and review of the literature. 1104 10

Plasma cell leukemia (PCL) is a very rare variant of multiple myeloma (MM) occurring in about 2% of newly diagnosed patients. Plasma cell leukemia may develop during the course of MM (secondary PCL) or it can occur without any prior sign of MM (primary PCL). We report a case of aggressive primary PCL with unusual clinical, cytogenetic and molecular features. A 36-year-old male patient was first seen because of fever and bone pain. On the skin of his chest, back, abdomen, and palpebras, there were nodular infiltrations resembling urticaria. White blood cell count was 10.8 x 10(9)/l with 41% plasmacytes. Bone marrow aspiration was hypercellular, 93.5% of cells were atypical plasmacytes and plasmablasts. The cytogenetic analysis of G-banded chromosomes in bone marrow cells yielded the trisomy 8. The skin biopsy specimen showed intensive infiltrates of uninucleated blastic cells similar to those found in the bone marrow. Immunophenotyping of bone marrow and skin neoplastic cells showed CD45+, CD45Ro+, CD68+, CD38+ and cytoplasmic kappa light chain +. The neoplastic cells stained negatively for lambda light chain, CD3, CD20, CD30, EMA, CD15, CD34, CD56 and factor VIII. The pattern of IgL genes rearrangement in the bone marrow aspirate, peripheral blood mononuclear cells, and skin specimens was examined by PCR analysis. All studied specimens showed three different IgK gene configurations suggesting that the neoplastic cells originated as a result of oligoclonal lymphoproliferation process. The patient received two courses of VAD (vincristine, doxorubicin, dexamethasone) without improvement and three courses of CHOP with only temporary stabilization of the disease. He died 5 months after the diagnosis of PCL because of disease progression and pneumonia.
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PMID:Aggressive primary plasma cell leukemia with skin manifestations, trisomy 8 and molecular oligoclonal features. 1214 88