Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 47-year-old white male developed massive hepatosplenomegaly, a pleural effusion, leucocytosis, and a left parasternal mass following a relatively symptom-free persistent hypereosinophilia for about 5 years. Bone marrow aspiration and biopsy and peripheral blood differential showed eosinophilia and a shift to the left with immature cells. A high serum B12 vitamin level and low LAP activity were found. Biopsy of the soft tissue mass revealed a granulocytic sarcoma (chloroma) with a hyperdiploid karyotype (49,XY, + 10, + 15, + 19,3q-), whereas the bone marrow cells had a normal male karyotype. The patient responded temporarily to chemotherapy but eventually developed CNS leukemia and went on to terminate in a frank blastic phase. This case illustrates hypereosinophilia and a myeloproliferative syndrome characterized by a somewhat indolent chronic course evolving into "eosinophilic leukemia" and granulocytic sarcoma, CNS involvement by leukemic cells and, finally, blastic transformation. It is possible that this case represents a variant of Ph1-negative CML to which the term "chronic eosinophilic leukemia" could be justifiably applied.
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PMID:Chromosomes and causation of human cancer and leukemia. XXXIV. A case of "hypereosinophilic syndrome" with unusual cytogenetic findings in a chloroma, terminating in blastic transformation and CNS leukemia. 29 66

The incidence of hematologic malignancies and their extranodal manifestations is continuously increasing. Previously unsuspected hepatic involvement in hematologic malignancies such as Hodgkin disease and non-Hodgkin lymphoma, posttransplant lymphoproliferative disorder, myeloid sarcoma (chloroma), multiple myeloma, Castleman disease, and lymphohistiocytosis may be seen by radiologists. Although the imaging features of more common hepatic diseases such as hepatocellular carcinoma, metastases, and infection may overlap with those of hepatic hematologic malignancies, combining the imaging features with clinical manifestations and laboratory findings can facilitate correct diagnosis. Clinical features that suggest a hematologic neoplasm as the cause of liver lesions include a young patient (<40 years of age), no known history of cancer, abnormal bone marrow biopsy results, fever of unknown origin, and night sweats. Imaging features that suggest hematologic malignancy include hepatosplenomegaly or splenic lesions, vascular encasement by a tumor without occlusion or thrombosis, an infiltrating mass at the hepatic hilum with no biliary obstruction, and widespread adenopathy above and below the diaphragm. Familiarity with the imaging features of hepatic hematologic malignancies permits correct provisional diagnosis and may influence therapeutic management. For example, when biopsy is performed, core biopsy may be needed in addition to fine-needle aspiration so that the tissue architecture of the neoplasm can be discerned. The predominant treatment of hematologic malignancies is chemotherapy or radiation therapy rather than surgery. Online supplemental material is available for this article.
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PMID:Hematologic malignancies of the liver: spectrum of disease. 2656 43