Extranodal NK/T-cell lymphoma (ENKTL), nasal type and aggressive NK cell leukemia are rare in Western World been less than 1% in USA to 8% in Asia among Non-Hodgkin's lymphomas. It is aggressive, with poor outcome and optimal treatment is unclear. A combination therapy that includes Peg-Asparaginase (SMILE) has been employed in young patients.
View Article and Find Full Text PDFChronic lymphocytic leukemia (CLL) is a malignant proliferation of monoclonal mature B-cells in peripheral blood. Leukemia cells can commonly spread from the blood to other sites such as the lymph nodes, liver, and spleen. However, contrary to T-cell lymphomas that can involve the skin, CLL metastasis to the skin is unusual and is rarely the first manifestation of systemic disease.
View Article and Find Full Text PDFIdentifying underlying bleeding diathesis that is amenable to medical therapy must be determined to provide timely treatment and minimize morbidity. Nasal bleeding is viewed as an annoyance by most who suffer from its episodes. However, it can at times be a baleful ailment that can compromise a patient's airway, breathing, and circulation, which can result in death.
View Article and Find Full Text PDFMucosa-associated lymphoid tissue (MALT) lymphomas are B-cell neoplasms that commonly affect the gastrointestinal (GI) tract, usually the stomach. In most cases, extranodal marginal zone lymphoma (ENMZL) is an indolent disease. Bone marrow involvement is common with MALT lymphoma accompanied by paraproteinemia; such involvement impels disease progression.
View Article and Find Full Text PDFIgM multiple myeloma is an exceedingly rare hematologic entity comprising only less than 0.5% of multiple myeloma cases. Given the rarity of this disorder, it makes it a challenge to differentiate from other more prevalent hematologic disorders like Waldenstrom macroglobulinemia.
View Article and Find Full Text PDFAggressive systemic mastocytosis is a rare hematologic neoplastic disease that presents with a poor prognosis and low survival rate. It typically manifests with symptoms associated to mast cell release of bioactive substances, causing anaphylaxis, flushing, autonomic and hemodynamic instability, gastric distress and headache. Moreover, more than 95% of cases are related to a mutation in codon 816 of the gene, located on human chromosome 4q12 which codes for a type III receptor tyrosine kinase.
View Article and Find Full Text PDFA man aged 69 years presented with acute right flank pain secondary to a hemorrhagic large adrenal tumor. En bloc resection was performed to repair the inferior vena cava. Immunoperoxidase levels in the tumor were positive for factor VIII and CD31 and negative for S100, protein Melan-A, CD34, synaptophysin, chromogranin, desmin, muscle specific actin, ETFA (EMA), KRT20 (CK20), CDX2, TTF1, LNPEP (PLAP), inhibin, ?-fetoprotein, CD30, hepatocyte paraffin, and aberrant expression of cytokeratin 7 and pankeratin.
View Article and Find Full Text PDFProlymphocytic leukemias (PLLs) are rare mature lymphoid disorders of B- and T-cell subtypes with distinct features and an aggressive clinical course. PLL represents only 2% of all mature lymphocytic leukemias in adults. T-PLL represents 20% of all PLLs cases.
View Article and Find Full Text PDFParoxysmal nocturnal hemoglobinuria (PNH) is a non-malignant, acquired clonal hematopoietic stem cell disease that can present with bone marrow failure, hemolytic anemia, smooth muscle dystonias, and thrombosis. We present a case of a 32 year-old-female, G2P2A0 with no past medical history of any systemic illnesses who refers approximately 2 months of progressively worsening constant heartburn with associated abdominal discomfort. CBC showed leukopenia (WBC 2.
View Article and Find Full Text PDFSplenic artery aneurysms (SAA) are a rare life threatening clinical diagnosis. We present a case of a young Hispanic woman with an aneurysm of the middle branch of the splenic artery and active leakage. The defect was embolized with complete resolution of the retroperitoneal bleeding.
View Article and Find Full Text PDFMadelung's disease is an extremely rare disorder of unknown etiology characterized by multiple, non-encapsulated, infiltrative lipomas located symmetrically on the trunk, neck, and proximal parts of the limbs. Approximately 200 patients have been reported in the medical literature. In this case report we present an extremely unusual case of multiple symmetric lipomatosis compatible with Madelung's disease.
View Article and Find Full Text PDFKaposi's sarcoma is a rare malignancy requiring infection with human Herpes virus for development. We report a case of a 76-year-old immunocompetent male with recurrent leg cellulitis. The cellulitis eventually developed into a non-healing ulcer and a palpable nodule consistent with nodular Kaposi's sarcoma.
View Article and Find Full Text PDFA 64 year-old Hispanic male patient presented to our institution with a three-month history of frontal headaches, reduced vision, retroorbital pain, photophobia, sinus congestion, bloody nasal discharge, and decreased audition in the left ear. The diagnosis of metastatic lymphoepithelioma to the orbit was made based on clinical history, histopathological examination of an orbital biopsy, and imaging studies. Lymphoepithelioma rarely develops in Hispanic populations.
View Article and Find Full Text PDFNeuroendocrine carcinomas of the colon and rectum comprise fewer than 1% of all colorectal cancers. These aggressive tumors generally have a poor prognosis compared to that associated with colorectal adenocarcinoma. We describe herein the case of a 68-year-old female presenting with a bleeding rectal mass involving the anal canal, which case was associated with hyponatremia due to inappropriate serum levels of antidiuretic hormone.
View Article and Find Full Text PDFBol Asoc Med P R
December 2012
Choledochal cyst type II, is an extremely rare cause of portal hypertension, severe pancytopenia in an adult patient, and a complication of long-standing disease. We present an uncommon cause of portal hypertension in a young female patient secondary to an obstructive choledochal cyst type II provoking massive splenomegaly and severe pancytopenia. A high level of clinical suspicion is important due to its high mortality rate if it remains undiagnosed.
View Article and Find Full Text PDFWe report a case of a young female patient presenting with a high serum beta-HCG levels, amenorrhea, nausea and anemia which mimicked pregnancy followed by upper gastrointestinal bleeding. A gastric tumor was shown on endoscopy. Histopathologic evaluation revealed Primary Gastric Choriocarcinoma (PGC).
View Article and Find Full Text PDFThis case report describes the clinical presentation, imaging findings and pathologic features of a rare aggressive breast tumor in a pre-menopausal woman, namely primary angiosarcoma. Recognition of this extremely rare entity is needed to make an early diagnosis, institute early therapy and eventually improve patient's survival.
View Article and Find Full Text PDFWe report the case of a female patient with an incidental finding at routine mammography evaluation which consisted of a benign spindle cell tumor, namely Breast Myofibroblastoma. It is arranged in fascicles with interspersed broad bands of hyalinized collagen with variable immunohistochemical reactivity to desmin, vimentin, smooth muscle actin and CD 34. It is usually not reactive to cytokeratins and S-100 as seen in the myoepitheliomas.
View Article and Find Full Text PDF