Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sixty-four patients with lymphoid lesions involving the lung were separated into three groups. In 32 patients, the predominant lymphoid cell population consisted of small, mature-appearing round lymphocytes with or without plasmacytoid features. This group, designated small lymphocytic proliferation (SLP), represents a heterogeneous group of pulmonary lymphocytic lesions including small lymphocytic lymphoma, lymphocytic interstitial pneumonia, and lymphoid hyperplasia (pseudolymphoma). Thirteen SLP patients were identified as having small lymphocytic lymphoma on the basis of monoclonality, progressive disease in other sites, or both. This group was morphologically identical to the remainder of the SLP patients, except for a higher incidence of plasmacytoid features (P = 0.003) and a greater degree of mast cell infiltration (P less than 0.05). Four of these 13 patients subsequently developed an aggressive large cell lymphoma resulting in death in three patients. The median survival for all of the SLP patients has not yet been reached. Patients in whom a monoclonal cell population could be established showed a slightly worse prognosis of borderline statistical significance (P = 0.09); however, the presence of a serum monoclonal gammopathy conveyed a significantly worse prognosis (P = 0.003). The remaining two groups of patients had various forms of malignant lymphoma other than the small lymphocytic type. One group of 12 patients, designated as having presumed primary lymphoma limited to one or both lungs (PL), had a prolonged course with a median survival of 117 months. The remaining 20 patients had disseminated lymphoma also involving lung (DL); DL patients had a shorter median survival of 33 months.
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PMID:Pulmonary lymphomas and other pulmonary lymphoid lesions. A clinicopathologic and immunologic study of 64 patients. 383 61

Patients with systemic mastocytosis (SM) may acquire an associated hematologic non-mast cell (MC)-lineage disease (AHNMD). In most cases, a myeloid neoplasm is diagnosed, whereas the occurrence of a lymphoproliferative disease is an extremely rare event. We report on a patient with indolent SM associated with small lymphocytic lymphoma (SLL). The patient presented with lymphadenopathy, maculopapular exanthema, and elevated serum tryptase. The bone marrow biopsy showed focal MC aggregates together with SLL. As assessed by immunostaining, neoplastic MC were found to exhibit CD117 and CD25 but did not display CD5 or CD20, whereas SLL cells were found to coexpress CD5 and CD20 but did not express MC antigens. The KIT mutation D816V was detected in sorted CD34(+) cells and unfractionated marrow cells but not in CD5(+) SLL cells, confirming the coexistence of 2 distinct neoplasms.
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PMID:Indolent systemic mastocytosis associated with atypical small lymphocytic lymphoma: a rare form of concomitant lymphoproliferative disease. 1844 46

BACKGROUND Systemic mastocytosis with an associated hematologic non-mast cell lineage disease is a rare entity, and the majority of systemic mastocytosis cases are associated with myeloid neoplasm. Lymphoproliferative disorders are less commonly associated with systemic mastocytosis and a few cases of systemic mastocytosis associated with chronic lymphocytic leukemia have been described in the literature. CASE REPORT We present a case of indolent systemic mastocytosis associated with small lymphocytic lymphoma. The bone marrow biopsy demonstrated mast cells in the form of clusters and perivascular distribution on immunohistochemistry for tryptase, CD2, and CD25 markers. In addition, 30% involvement by small lymphocytic lymphoma was discovered in the form of interstitial lymphoid aggregates composed of small lymphocytes. Flow cytometry showed B-cells positively stained for CD19, CD20, CD5, CD23, and kappa light chains, and the CD38 expression was <5%. CONCLUSIONS In systemic mastocytosis with an associated hematologic non-mast cell lineage disease, the combination of systemic mastocytosis associated with small lymphocytic lymphoma is rare and the management strategy follows the principle of treating the two entities individually as if they are not related. Clinical surveillance is indicated for indolent systemic mastocytosis and low-risk small lymphocytic lymphoma to monitor for disease progression.
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PMID:Systemic Mastocytosis in Association with Small Lymphocytic Lymphoma. 2897 Apr 67