Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 59-year-old man with myelodysplastic syndrome who was hospitalized for evaluation of fever and generalized fatigue had elevated levels of C-reactive protein and pancytopenia. A search for a site of infection and empiric treatment with antibiotics were unsuccessful. Over 5 to 6 weeks right upper quadrant pain and rebound tenderness developed. Sonographic Murphys sign was present. Computed tomography showed thickening of the gallbladder wall, and repeated ultrasonography demonstrated changes consistent with cholecystitis. Open cholecystectomy was performed as an emergency procedure. Macroscopically the resected gallbladder showed an edematous and thickened wall. Histopathologic examination revealed transmural infiltration by atypical mononuclear cells with distinct nuclei. The cells showed immunohistochemical staining for CD15, indicating myeloid lineage. By 10 days after surgery, counts of leukocytes and leukoblasts had markedly increased, reaching 36,700/microL and 76.0%, respectively. The blast crisis was thought to indicate progression from myelodysplastic syndrome to leukemia. The patient died of progressive disease 12 days after surgery. We have described a rare case of acute cholecystitis caused by infiltration of immature myeloid cells to the gallbladder. An acute abdomen complicating hematologic disorders is life-threatening and requires prompt and appropriate treatment.
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PMID:Cholecystitis caused by infiltration of immature myeloid cells: a case report. 1664 35

Sclerosing extramedullary hematopoietic tumor (SEMHT) is a rare disease that was originally named fibrous hematopoietic tumor or myelosclerosis. The process typically occurs in patients with a history of chronic myeloproliferative disorders and may afflict the skin, lung, breast, gastrointestinal tract, breast, kidney, lymph node, and thyroid gland. In this article, we report the case of a 37-year-old female with more than 5 years history of acute B-cell lymphoblastic leukemia who presented with right upper quadrant pain and tenderness. Computed tomography scan showed multiple new ring-enhancing low-density lesions within the right and left hepatic lobes. A computed tomography-guided liver core biopsy was performed on one of the new liver lesions and showed extramedullary hematopoiesis with atypical megakaryocytes and sinusoidal capillarization with associated fibroblastic proliferation. Numerous atypical megakaryocytes were scattered in the background of haphazard collagen deposition. No significant blasts or a leukemic process were identified. Bone marrow aspiration and biopsy showed extensive reticulin fibrosis (MF-3), trilineage dysplasia, increased blasts (10% to 19%), and hypercellularity (close to 100%), which was consistent with MDS-EB-2. Cytogenetics was reported as follows: 44~46,XX,-3,add(3)(p13),-5,-6,-7,17,del(17)(p12),+1~5mar[cp9]/46,XX. Molecular analysis was negative for both JAK2 V617F and CALR exon 9 activating mutations. In summary, we contributed a new case of SEMHT diagnosis in a synchronous presentation with poor clinical evolution associated chromosome 7 deletion and intact JAK2 and CALR exon 9. Care should be taken when diagnosing intraabdominal and retroperitoneal soft tissue masses with the history of hematological disorders.
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PMID:Sclerosing Extramedullary Hematopoietic Tumor: A Case Report. 3291 87