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)

Kikuchi-Fujimoto disease also known as histiocytic necrotizing lymphadenitis is a rare cervical inflammatory lymphadenitis that is most commonly seen in young Asian women. It is mainly characterized by lymphadenopathy, hepatosplenomegaly, fever, nocturnal sweats, myalgia, weight loss, and arthralgia, and commonly follows a self-limited course. The differential diagnosis is challenging as many other conditions such as malignant lymphoma, metastatic disease, tuberculosis and infectious lymphadenopathies can present in a similar way. We present an unusual case of Kikuchi-Fujimoto disease masquerading as metastatic papillary carcinoma of the thyroid. A 30-year-old young female presented, 2 months post-partum, with complaints of neck pain and fever. A computed tomography scan showed enlarged right-sided lymph nodes and a thyroid nodule. Subsequent biopsy of a thyroid nodule revealed papillary thyroid carcinoma and reactive inflammation in one of the lymph nodes. She underwent an elective total thyroidectomy, central node dissection and a right modified lymph node dissection for enlarged lymph nodes. Her recovery was uneventful and the pathology report was consistent with a papillary carcinoma of the thyroid with one lymph node positive for metastatic disease and several other lymph nodes showing histiocytic necrotizing lymphadenitis. This coexistence of Kikuchi-Fujimoto disease with localized metastatic papillary thyroid cancer is unusual and presents an interesting, challenging, and complex management dilemma.
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PMID:Kikuchi-Fujimoto Disease Masquerading as Metastatic Papillary Carcinoma of the Thyroid. 2606 Mar 88

A 52 year-old male presented with neck pain after undergoing thyroidectomy for a goiter three weeks prior which was complicated by a neck hematoma requiring evacuation. Computed tomography (CT) scan showed a neck hematoma requiring evacuation and he received desmopressin with cessation of bleeding. Coagulation studies were normal. He returned eighteen months later with severe oral mucosal bleeding after a dental procedure and required transfusions with red blood cells, platelets, and fresh frozen plasma (FFP) in addition to desmopressin, Humate-P, aminocaproic acid, and surgical packing. A comprehensive bleeding diathesis workup was normal. He was readmitted six months later due to abdominal pain and distention and found to have massive hepatosplenomegaly on CT. A new coagulopathy workup revealed prolonged INR to 1.5, corrected prothrombin time mixing study, and a low factor VII level (29%), suggesting acquired factor VII deficiency. A transjugular liver biopsy revealed extensive involvement by ALamyloidosis- Kappa type. He then developed a large right retroperitoneal hematoma which required multiple transfusions with FFP, cryoprecipitate, aminocaproic acid, and vitamin K with slight success. Hemorrhage was subsequently stabilized with recombinant factor VIIa administered every four hours which corresponded with correction of factor VII levels and PT and eventual cessation hemorrhage. Acquired factor VII deficiency causing severe coagulopathy was attributed to hepatic amyloidosis ALkappa subtype. We started treatment with bortezomib, dexamethasone, and cyclophosphamide, however, the patient succumbed to uncontrolled hemorrhage. Acquired factor VII deficiency is extremely rare and to our knowledge, this is the only known case of factor VII deficiency secondary to amyloidosis involving the liver.
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PMID:Acquired factor VII deficiency causing severe bleeding disorder secondary to AL amyloidosis of the liver. 3034 85