Unexpectedly high rates of venous thromboembolic events (VTE) induced by highly effective immune modulating drugs thalidomide and lenalidomide for treatment of multiple myeloma have focused attention on the incidence and underlying pathophysiology of VTE in patients with plasma cell dyscrasias, and on thromboprophylaxis approaches. While bleeding complications are relatively uncommon in the patients with lymphoproliferative disorders, acquired von Willebrand syndrome, typically occurring in the patients with monoclonal gammopathy of unknown significance, and acquired coagulopathies associated with primary amyloidosis can present with haemorrhagic complications and both are challenges to the management. This review highlights these important haemostasis-related complications of plasma cell dyscrasias and provides an overview of other uncommon bleeding and thrombotic events that can affect diagnosis and therapeutic management of clonal plasma cell disorders. Due to the infrequency of most these haemostasis complications, available information is typically based on retrospective cases or series analysis.
Hemorrhage ; etiology ; pathology ; therapy ; Humans ; Paraproteinemias ; complications ; pathology ; Thrombosis ; etiology ; pathology ; prevention & control

Enterovirus A, Human ; Enterovirus Infections ; pathology ; Female ; Hemorrhage ; etiology ; virology ; Humans ; Infant ; Male ; Pulmonary Edema ; etiology ; virology

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder that has three major features: multiple neural tumors, cafe-au-lait spots, and pigmented iris hamartomas (Lisch nodules). The purpose of this case report is to advise physicians of the danger associated with the progression of fast-onset massive hemorrhage to hemodynamic instability, which mandates rapid treatment to prevent the development of a life-threatening condition. A 64-yr-old woman with NF-1 was admitted to the Emergency Department (ED) because of a rapidly growing, 10x5x3 cm-sized mass on the left back area. She had previously undergone surgery for a large subcutaneous hematoma, which had developed on her right back area 30 yr before. She became hemodynamically unstable with hypotension during the next 3 hr after admission to ED. Resuscitation and blood transfusion were done, and the hematoma was surgically removed. The mass presented as a subcutaneous, massive hematoma with pathologic findings of neurofibroma. We report a case of NF-1 that presented as recurrent, massive, subcutaneous hemorrhage on the back region combined with hypovolemic shock.
Diagnosis, Differential ; Female ; Hematoma/etiology/pathology ; Hemorrhage/*etiology/pathology ; Humans ; Middle Aged ; Neurofibromatosis 1/*complications/pathology ; Recurrence ; Skin Diseases/*etiology/pathology ; Tomography, X-Ray Computed

Acute post-streptococcal glomerulonephritis (PSGN) is characterized by an abrupt onset of edema, hypertension, and hematuria. Life-threatening diffuse alveolar hemorrhage (DAH) is rarely associated with acute PSGN. There have been only two reported cases worldwide, and no case has been reported previously in Korea. Here, we present a patient who clinically presented with pulmonary-renal syndrome; the renal histology revealed post-infectious glomerulonephritis of immune complex origin. A 59-yr-old woman was admitted with oliguria and hemoptysis two weeks after pharyngitis. Renal insufficiency rapidly progressed, and respiratory distress developed. Chest radiography showed acute progressive bilateral pulmonary infiltrates. The clinical presentation suggested DAH with PSGN. Three days after treatment with high-dose steroids, the respiratory distress and pulmonary infiltrates resolved. Electron microscopy of a renal biopsy specimen sample revealed diffuse proliferative glomerulonephritis with characteristic subendothelial deposits of immune complex ("hump''). The renal function of the patient was restored, and the serum creatinine level was normalized after treatment.
Biopsy ; Female ; Glomerulonephritis/*etiology ; Hemorrhage/*etiology ; Humans ; Kidney/pathology ; Lung Diseases/etiology ; Middle Aged ; *Pulmonary Alveoli ; Streptococcal Infections/*complications

Inflammatory fibroid polyp occurs very rarely in the jejunum and gastrointestinal bleeding as an initial manifestation of inflammatory fibroid polyp has not been reported. We report a case of a jejunal inflammatory fibroid polyp presenting with melena for 10 days. Upper gastrointestinal endoscopic examination was negative for any active bleeding lesions and abdominal angiography failed to localize the bleeding site as well. In contrast, computed tomography of the abdomen demonstrated a segmental wall thickening of the jejunum with a tumor-like mass lesion associated with dense contrast enhancement. Consistent with this, technetium 99m red blood cells scintigraphy exhibited red cell pooling at the right upper quadrant. On exploratory laparotomy, there was an active bleeding from the site of the jejunal tumor and a segmental resection was performed. Histologically, the tumor lesion of the jejunum was consistent with inflammatory fibroid polyp. Thus, we conclude that the tumor lesion was a cause of the gastrointestinal bleeding.
Adult ; Gastrointestinal Hemorrhage/*etiology ; Humans ; Intestinal Polyps/diagnosis/*pathology ; Jejunal Diseases/diagnosis/*pathology ; Male

Esophageal and Gastric Varices ; diagnosis ; etiology ; pathology ; Hemorrhage ; Humans ; Liver Cirrhosis ; complications ; pathology

Adolescent ; Diagnosis, Differential ; Diverticulum, Stomach ; complications ; diagnosis ; pathology ; Gastrointestinal Hemorrhage ; diagnosis ; etiology ; pathology ; Gastroscopy ; Humans ; Male

The gastrointestinal stromal tumor (GIST) is a mesenchymal tumor of the digestive tract showing differentiation along the line of interstitial cell of Cajal. The most GISTs in the stomach generally show the appearance of submucosal tumors. It is rare for GISTs to appear as a pedunculated polypoid lesion on endoscopy. We experienced a case of a 51-year-old man who had a pedunculated polypoid GIST. He was admitted to our hospital for nausea, vomiting, melena and severe anemia (hemoglobin 3.4 g/dL, hematocrit 10.8%). An upper endoscopy showed gastroduodenal intussusception due to a pedunculated polypoid mass. This report presents a rare case of endoscopically proven gastroduodenal intussusceptions due to pedunculated polypoid GIST in the stomach.
Duodenal Diseases/etiology/*pathology ; Gastrointestinal Hemorrhage ; Gastrointestinal Neoplasms/complications/*pathology ; Gastrointestinal Stromal Tumors/complications/*pathology ; Gastroscopy ; Humans ; Intussusception/etiology/*pathology ; Male ; Middle Aged ; Tomography, X-Ray Computed

No abstract availble.
Adult ; Arteries/abnormalities ; Diagnosis, Differential ; Endoscopy, Gastrointestinal ; Gastrointestinal Hemorrhage/*etiology/pathology ; Humans ; Intestinal Mucosa/blood supply/pathology ; Jejunal Diseases/*etiology/pathology ; Jejunum/*blood supply/pathology ; Male

Aged, 80 and over ; Amyloid beta-Peptides ; metabolism ; Brain ; pathology ; Cerebral Amyloid Angiopathy ; complications ; metabolism ; pathology ; Cerebral Hemorrhage ; etiology ; pathology ; Consciousness Disorders ; etiology ; pathology ; Humans ; Male