Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Severe splenomegaly was found during routine examination of a clinically normal 7-yr-old male Asian small clawed otter. The spleen and three enlarged splenic lymph nodes were immediately removed. The spleen weighed 310 g (approximately 8% of body weight). The spleen and resected lymph nodes were diffusely infiltrated by coalescing sheets of neoplastic lymphocytes that occasionally surrounded remnants of preexisting lymphoid follicles. Immunohistochemical confirmation of B lymphocyte origin and microscopic pattern were consistent with primary splenic marginal zone lymphoma (MZL) with metastasis to the splenic lymph nodes. The otter received no additional treatment and survived for 16 mo following splenectomy. Necropsy confirmed metastasis to multiple abdominal and extra-abdominal lymph nodes, liver, and kidney, and renal failure related to glomerulosclerosis. The prolonged survival in this otter is typical for MZL, an indolent form of B-cell lymphosarcoma that spreads slowly to the abdominal and extra-abdominal lymph nodes.
 ...
PMID:Splenic marginal zone lymphoma in an Asian small-clawed otter (Aonyx cinerea). 2531 52

Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm. We herein describe the case of a 41-year-old woman who was admitted with nephrotic syndrome (NS) and severe neutrophilia and underwent a splenectomy due to splenomegaly. Peripheral blood tests revealed a Janus kinase 2 (JAK2) V617F mutation without the Philadelphia chromosome, BCR-ABL fusion transcripts, or FIP1 L1-platelet-derived growth factor (PDGF)a. A kidney biopsy showed focal segmental glomerulosclerosis (FSGS) with interstitial neutrophil infiltration and with a JAK2 V617F mutation. Hydroxyurea was initiated for first three months, followed by hydroxyurea plus interferon, and a subsequent improvement in leukocytosis and completely remission of FSGS-NS was immediately noted. This is the first case reported in which NS was related to CNL.
...
PMID:Nephrotic syndrome related to chronic neutrophilic leukemia. 2536 11

A rare case of lethal idiopathic plasmacytic lymphadenopathy (IPL) with polyclonal hyperimmunoglobulinemia with chronic renal failure is described. A 40-year-old woman who had suffered from upper airways disease was admitted to the Emergency Room with acute renal failure and hypergammaglobulinemia. She developed pericardial effusion, a pruritic rash, splenomegaly and fell into a coma after 6 days. Multiple myeloma, infection, collagenopathy, and coagulopathy were ruled out. Finally, a form of malignant hypergammapathy was suspected. At autopsy, lymph nodes were infiltrated by polyclonal plasma cells and lymphocytes, with erythrophagocytosis features; immunohistochemistry confirmed the plasma cells (CD138+), with a prevalence of kappa-positive cells, B (CD20+) and T (CD3+) cells. Kidneys showed advanced glomerulosclerosis containing similar infiltrates. The cause of death was IPL with polyclonal hypergammaglobulinemia and advanced renal failure. Similar cases are unusual, and possible medical liability associated with failure to diagnose and treat idiopathic plasmacytic lymphadenopathy deserves discussion and further studies.
 ...
PMID:A Case of Lethal Idiopathic Plasmacytic Lymphadenopathy with Polyclonal Hypergammaglobulinemia: A Medical Challenge for the Forensic Pathologist. 2736

POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. We present an atypical case of POEMS syndrome with subclinical polyneuropathy, splenomegaly, hypogonadism, and myeloma of immunoglobulin G kappa type. The typical osteosclerotic lesions and skin lesions were absent, and vascular endothelial growth factor (VEGF) level was normal. Our patient also had chronic kidney disease due to focal segmental glomerulosclerosis. The patient died due to multisystem complications nine months after presenting with acute on chronic renal failure. POEMS syndrome is an uncommon, potentially fatal paraneoplastic syndrome presenting with plasma cell neoplasm and multisystem involvement. Despite the normal VEGF level, our patient was diagnosed to have possible POEMS syndrome due to other clinical manifestations. It may be reasonable to expand the diagnostic criteria of POEMS syndrome if further atypical cases are reported in the future.
 ...
PMID:An atypical presentation of POEMS syndrome with IgG kappa type M protein and normal VEGF level: Case report and review of literature. 2989 39


<< Previous 1 2