[A case of blastic plasmacytoid dendritic cell neoplasm involving the skin].

A 81 year-old male patient visited our hospital on February, 2011 because he found the subcutaneous tumor on his back. The dermatologist performed skin biopsy and found that large atypical cells diffusely proliferated in the sub-epidermal and subcutaneous tissue. Two month later, we diagnosed him as blastic plasmacytoid dendritic cell tumor, as the blastic cells in the lymph node were CD4+, CD56+, CD3-, CD5-, CD20-, CD138-, MPO-, granzyme B-, TCL1+. None of the gene rearrangements of T-cell receptor and immunoglobulin was negative in the lymph node. The tumor was relapsed after 3 courses of COP therapy and the patient failed to respond to the salvage therapies such as CEPP and FMD therapy. As this tumor is rare and there are no standard regimens, we reviewed the past case series and discussed about the pathogenesis, clinical course and treatment options of this tumor in this article.

[Two severe tetanus cases infected in an urban area of Kawasaki City].

We report two cases of severe tetanus infection. Case 1: A 73-year-old non-vaccinated man who fell in a local park developed a wound on the left little finger. The wound was debrided and a tetanus toxin shot given on day 4 following the injury. He developed trismus on day 6 requiring deep sedation and mechanical ventilation in the intensive care unit (ICU), with human anti-tetanus immune globulin (TIG) and antibiotics administered. Despite a very severe autonomic dysfunction, he recovered and was discharged mobile after 2 months of rehabilitation. Case 2: A 37-year-old woman fully vaccinated against tetanus in her childhood had apparently had booster vaccine for at least 20 years and was being treated for hyperthyroidism with thiamazole. She sustained two lacerations on the fingers of her right hand in her backyard. She noticed difficulty in opening her mouth on day 3 following the injury and was seen on day 7, for high fever and difficulty in speaking. She was diagnosed clinically as having tetanus and underwent wound debridement, and a shot of tetanus toxin, TIG, and antibiotics. On hospital admission day 2, she developed spasms and her blood pressure dropped drastically. She died the next day due to endotoxin shock caused by other bacteria. C. tetani is widely distributed in Japan, and these cases underscore the importance of maintaining adequate tetanus antibody levels through booster administration every 10 years in immune adults and appropriate post-exposure treatment with tetanus toxin and/or prophylactic TIG administration.

Primary diffuse large B-cell lymphoma of the bladder.

Primary lymphoma of the bladder is quite rare; primarily, it is extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma). There is only one case report of primary diffuse large B-cell lymphoma (DLBCL) of the bladder, accompanied by diffuse wall thickening of the bladder. Here, we report a second case of primary DLBCL of the bladder in a 75-year-old woman patient, whose initial presentation was acute renal failure. Three courses of R-CHOP chemotherapy were effective to treat acute renal failure caused by post-renal obstruction and to attain clinical remission.

Paravertebral extramedulary hematopoiesis arising with osteopetrosis tarda.

Extramedullary hematopoiesis predominantly occurs in the liver, spleen, and lymph nodes with hemolytic anemia. Occurrence with osteopetrosis tarda in the paravertebral region is very rare. We discuss the examination of the third known case of paravertebral extramedullary hematopoiesis arising with osteopetrosis tarda.

[A case of probable bacterial translocation-associated sepsis in healthy adult].

We report a patient with bacterial translocation-associated sepsis who was healthy and did not have any related-background. The 57-year-old male had been well until 16 hours before admission, when nausea and vomiting gradually developed and increased in intensity. In the morning of May 22, 2002, he had shaking chills, temperature of 38.6 degrees C and watery diarrhea, and was admitted to Kawasaki Municipal Hospital. On admission, temperature was 40.7 degrees C but otherwise physical examination revealed no particular abnormality. Laboratory data showed total white blood cells of 28,400/microliter, platelet count of 130,000/microliter, creatinine of 2.0 mg/dl and C-reactive protein of 7.5 mg/dl. 1 g of cefmetazole was administered every eight hours. In the early morning of May 23, he suddenly went into shock. At that time, laboratory findings revealed total white blood cells of 33,700/microliter, platelet count of 65,000/microliter, C-reactive protein of 24.9 mg/dl, creatinine of 5.6 mg/dl and serum potassium concentration of 5.7 mEq/l. Gram positive cocci and gram negative rods were isolated from blood culture obtained on admission. Cefmetazole was changed to 1.5 g/day of imipenem/cilastatin sodium and 600 mg/day of clindamycin. In addition, hemodialysis and endotoxin removal with an adsorbent column using polymyxin B were performed. Bacteria detected in the blood on admission were identified as Klebsiela oxytoca and Enterococcus faecium. Imipenem/cilastatin sodium and clindamycin were continued for 13 days. The patient recovered fully and was discharged on June 11. This case suggests that bacterial translocation-associated sepsis might occur even in a hitherto healthy adult.

Anti-c-Mpl (thrombopoietin receptor) autoantibody-induced amegakaryocytic thrombocytopenia in a patient with systemic sclerosis.

Amegakaryocytic thrombocytopenia (AMT) associated with systemic sclerosis (SSc) has been described in several case reports, but the underlying mechanisms have not been identified. Here we describe a rare case of SSc accompanied by thrombocytopenia and megakaryocytic hypoplasia, in which autoantibody against thrombopoietin receptor (c-Mpl) was detected. A 61-year-old woman with limited SSc was admitted to our hospital because of severe thrombocytopenia (platelet count 0.2 x 10(4)/mm(3)) with gingival bleeding. Her bone marrow was hypocellular with absent megakaryocytes, consistent with AMT. Treatment with corticosteroids and intravenous immunoglobulin infusions resulted in an increased platelet count, and she sustained a remission over a 1-year period, with a platelet count averaging 10.0 x 10(4)/mm(3). Her serum was strongly positive for anti-c-Mpl antibody, and IgG fraction purified from her serum inhibited thrombopoietin-dependent cell proliferation in vitro. Our case report suggests that AMT in patients with SSc could be mediated by the anti-c-Mpl antibody, which functionally blocks an interaction between thrombopoietin and c-Mpl.