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

Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis, characterised by elevated serum IgG4 levels. AIP is associated with many other diseases, including retroperitoneal fibrosis, sclerosing cholangitis, and sialoadenitis. Here, we report an interesting case of a 45-year-old male who presented with haematemesis, melena, and fever, accompanied by hepatosplenomegaly, systemic lymphadenopathy, diffuse swelling of the pancreas, portal hypertension, and multiple enlarged retroperitoneal lymph nodes on abdominal computed tomography (CT). The patient did not have a history of viral hepatitis or cirrhosis. Laboratory testing revealed an elevated IgG (3000 mg/dL). He underwent surgery for uncontrolled active upper gastrointestinal bleeding. We found splenomegaly, with a plump pancreas and involved peripheral lymph nodes, so a splenectomy was performed, and the pancreatic tail and some of the lymph nodes were biopsied. All of the resected tissues were infiltrated by large numbers of IgG4-positive plasma cells. Therefore, this patient was diagnosed with AIP associated with portal hypertension, systemic lymphadenopathy, and splenomegaly. The patient received no other treatment after the splenectomy. By the 6-month follow-up, the patient had recovered, the serum IgG had decreased to normal, and enhanced CT showed a normal pancreas. We speculate that splenectomy may be a new method of treating AIP.
 ...
PMID:Regional portal hypertension, systemic lymphadenopathy, and splenomegaly associated with autoimmune pancreatitis. 2333 30

Multiple myeloma is a rare malignancy that exhibits a wide range of possible clinical presentations. In recent years, with the advent of stem cell transplantation, the prognosis of patients with multiple myeloma has been increasing. We searched the literature for reports of atypical myeloma presentations to aid clinicians in formulating differential diagnoses and to increase the number of cases diagnosed early. There have been a number of reports of early ocular symptoms, including, but not limited to, proptosis, optic neuropathy, vision loss, retinal hemorrhage, and detachment. Neurological presentations included cranial nerve palsies, vertigo related to cerebellar involvement, and diabetes insipidus related to pituitary involvement. Among gastrointestinal manifestations, there are a number of reports of multiple myeloma presenting as acute and chronic pancreatitis. Mesenteric ischemia due to amyloidosis, acute abdomen, and hepatosplenomegaly were also among reported presentations. When it comes to renal involvement, while acute renal failure and proteinuria are typical, there are reports of patients presenting with both nephritic and nephrotic forms of glomerular disease, as well as end-stage renal disease requiring dialysis. We believe that it is essential for clinicians to keep reporting atypical multiple myeloma presentations and consider it as a possible diagnosis in a patient with serious, atypical symptoms.
 ...
PMID:Uncommon Presentations of Multiple Myeloma. 3263 79