Prolonged Bone Marrow Failure With Monosomy 7 After Engraftment Failure Following Bone Marrow Transplantation

A patient with acute myelogenous leukemia developed prolonged bone marrow failure along with the monosomy 7 chromosome abnormality. The patient had undergone bone marrow transplantation with CD34+ selection following induction failure. However, she then suffered engraftment failure and long-term pancytopenia. Her white blood cell count gradually increased with supportive therapy including granulocyte colony-stimulating factor (G-CSF), and chromosomal analysis of bone marrow cells revealed an abnormal karyotype. Thirty months after the bone marrow transplantation we observed monosomy 7 together with the existing chromosomal abnormality in the patient's bone marrow cells. It has been reported that some patients with idiopathic and posthepatitis aplastic anemia develop clonal disorders such as myelodysplastic syndrome/acute myelogenous leukemia with monosomy 7. The findings in our case suggest that the appearance of monosomy 7 in patients with aplastic anemia may be caused by prolonged low-level hematopoiesis, with or without G-CSF stimulation.     

Vascular surgery using argatroban in a patient with a history of heparin-induced thrombocytopenia.

For patients with a history of heparin-induced thrombocytopenia (HIT) who undergo cardiac or vascular surgery, the optimal anticoagulation substitute for heparin has yet to be established. Recombinant hirudin has been recommended; however, this agent is unsuitable for patients with renal dysfunction. Argatroban was used in the present patient who had a history of HIT and renal dysfunction and required peripheral vascular surgery. Argatroban was easy to monitor and control, regardless of renal function, and has advantages over other anticoagulants for such patients.     

A case of endocapillary proliferative glomerulonephritis with macrophages phagocytosing monoclonal immunoglobulin lambda light chain

Multiple myeloma (MM) is a plasma‐cell neoplasm that can cause renal disorders. Renal lesions in MM can present with a very rare pathological manifestation involving a specific monoclonal immunoglobulin (Ig). We report the case of a 33‐year‐old woman who had edema, fatigue, elevated serum creatinine levels, hypoalbuminemia, and hypercholesterolemia. She had persistent hematuria and proteinuria lasting 3 years. Serum protein electrophoresis showed an M‐spike, and serum immunofixation demonstrated the presence of monoclonal IgG λ. She had proteinuria in the nephrotic range, and a monoclonal λ fragment was present on urine immunofixation. Renal biopsy showed proliferative glomerulonephritis with λ light chain and C3c deposition and inflammatory cell infiltration with CD68. Macrophage lysosomes contained λ light chains, suggesting their partial phagocytosis. She was diagnosed with symptomatic MM and was treated with bortezomib and dexamethasone and an autologous peripheral stem cell transplant conditioned with intravenous melphalan. She achieved a partial response with decreased serum monoclonal protein and improved renal function. This case may be categorized as a monoclonal gammopathy‐associated proliferative glomerulonephritis. The biopsy finding of partially phagocytosed Ig λ light chains by macrophages is very rare; this pathological condition is similar to crystal‐storing histiocytosis.     

A Superinfection of Salmonella typhi and Hepatitis E Virus Causes Biphasic Acute Hepatitis

A 47-year-old Japanese man was referred to our hospital because of a sustained high fever with diarrhea 12 days after a flight from India. Liver enzymes were elevated with rose spots, hepatosplenomegaly, relative bradycardia, and acute cholecystitis. A liver biopsy depicted the dense infiltration of lymphocytes and Kupffer cells in sinusoids and the granulomatous formation in the parenchyma. The liver damage was initially resolved with the administration of ceftriaxone for 16 days but flared up 1 week later. Laboratory tests yielded positive reactions for Salmonella typhi and hepatitis E virus RNA. The pathophysiological presentations of concurrent typhoid and type E hepatitis are discussed.     

Relationship between juxtapapillary duodenal diverticula and biliopancreatic disease--evaluation by endoscopic biliary manometry]

Endoscopic biliary manometry was performed to evaluate the motor activity of the sphincter of Oddi (SO) in 28 patients, 13 with juxtapapillary duodenal diverticula (8 with biliopancreatic disease) and 15 without diverticula (10 with biliopancreatic disease). So pressure and the rate of irregular wave pattern of phasic contraction in patients with diverticula were significantly higher than those in patients without diverticula. Especially all the patients with both diverticula and biliary stones had motor disorders of the SO. The patients with diverticula were also divided into three groups by endoscopic findings for the location of diverticular; The papilla of Vater was located close to diverticula (within about 4 cm) in group A, on the edge of diverticula in group B, in the diverticula in group C. Diverticula could have more direct effect on the motor activity of the SO in group C. Those results suggest juxtapapillary diverticula have close relationship with the motor activity of the SO and biliopancreatic disease, especially in cases which the papilla of Vater located in the diverticula. We conclude that the motor disorders of the SO might be responsible for biliopancreatic disease in patients with juxtapapillary diverticula.     

Spontaneous Rupture of Hepatocellular Carcinoma: A Review of 172 Japanese Cases

The spontaneous rupture of a primary hepatocellular carcinoma (HCC) accounts for 10% mortality of HCC patients in Japan. Because this problem occurs much less frequently in western countries, it is often difficult for clinicians practicing in such countries to decide upon the best course of action during the crisis accompanying the spontaneous rupture of a HCC. In an effort to advance the general knowledge of this disease and clarify a selection for its treatment, we review 172 cases of spontaneous rupture of a HCC reported in the Japanese literature. The chief complaint of the patients was sudden epigastralgia or right hypochondriac pain. Abdominal paracentesis was positive in 86% of the patients. Liver failure was the cause of death in 42% of the patients. Therefore, it is important to evaluate liver reserve quickly. In addition, emergency hemostatic procedures must be implemented to avoid hemorrhagic shock. Although two-stage hepatectomy was performed in only 12% of the cases, these had the highest survival rates. Consequently, this is the procedure of choice for the treatment of spontaneous rupture of a HCC.