Defective interleukin-1 production in a familial monocyte disorder with a combined abnormality of mobility and phagocytosis-killing.

Monocytes in a familial monocyte disorder, a recently recognized primary immunodeficiency syndrome, with impaired phagocytic functions were studied for their ability to produce interleukin 1 (IL-1) as well as the surface property. Monocytes from two children (siblings) with the disorder possessed CD11b, CD13, CD14, CD33, Ia and LFA-1/Mac-1/p150,95 beta subunit antigens as determined by flow cytometry. Electron microscopic cytochemistry showed that the monocytes had surface glycoproteins reactive with four representative lectins. The IL-1 production by monocytes was assayed in the two patients and compared with that in six children with primary immunodeficiency syndromes and some monocyte abnormalities; three had congenital neutropenia, two had hyper-IgE syndrome, and one had defective monocyte chemotaxis. Monocyte culture supernatants were prepared with stimulation by lipopolysaccharide or silica, and their IL-1 activity was measured by the mouse thymocyte-proliferation assay. The patients' monocytes were defective in IL-1 production: the values were less than 1.0% of the control monocyte values (n = 12) and were in contrast with those of congenital neutropenia monocytes of 186.2% to 204.3%. These results demonstrate a familial monocyte disorder which is characteristic among the immunodeficiency syndromes with regard to the defective IL-1 production and the impaired phagocytic functions.     

Invasive fungal infection following reduced-intensity cord blood transplantation for adult patients with hematologic diseases.

Invasive fungal infection (IFI) is a significant complication after allogeneic hematopoietic stem cell transplantation (HSCT); however, we have little information on its clinical features after reduced intensity cord blood transplantation (RICBT) for adults. We reviewed medical records of 128 patients who underwent RICBT at Toranomon Hospital between March 2002 and November 2005. Most of the patients received purine-analogbased preparative regimens. Graft-versus-host disease (GVHD) prophylaxis was a continuous infusion of either tacrolimus 0.03 mg/kg or cyclosporine 3 mg/kg. IFI was diagnosed according to the established EORTC/NIH-MSG criteria. IFI was diagnosed in 14 patients. Thirteen of the 14 had probable invasive pulmonary aspergillosis and the other had fungemia resulting from Trichosporon spp. Median onset of IFI was day 20 (range: 1-82), and no patients developed IFI after day 100. Three-year cumulative incidence of IA was 10.2%. Four of the 13 patients with invasive aspergillosis (IA) developed grade II-IV acute GVHD, and their IA was diagnosed before the onset of acute GVHD. The mortality rate of IFI was 86%. Multivariate analysis revealed that the use of prednisolone >0.2 mg/kg (relative risk 7.97, 95% confidence interval 2.24-28.4, P = .0014) was a significant risk factor for IA. This study suggests that IFI is an important cause of deaths after RICBT, and effective strategies are warranted to prevent IFI.     

Epidermoid cyst derived from an accessory spleen in the pancreas. A case report with literature survey.

A rare case of splenic epidermoid cyst (SEC) of the pancreas discovered in a 32-year-old Japanese female is reported. The lesion, 5 x 6 cm in size including caseous material and serous fluid in the lumen, was discovered by ultrasonography and computed tomography at the tail of the pancreas and was easily removed. Histopathologically, the cystic wall consisted of three components: the inside was lined by mature squamous epithelium with keratinization, the middle layer consisted of splenic pulp with a sinus structure, and the peripheral layer was dense fibrous connective tissue in which some involutional pancreatic ducts and islets were recognized. The literature about SEC of the pancreas is discussed in comparison with other types of epidermoid cyst including lymphoepithelial cyst and dermoid cyst in the pancreas.     

Cytomegalovirus infections following umbilical cord blood transplantation using reduced intensity conditioning regimens for adult patients.

Cytomegalovirus (CMV) infection is a major complication after allogeneic hematopoietic stem cell transplantation (Allo-HSCT); however, we have little information on the clinical features of CMV reactivation after cord blood transplantation using reduced-intensity regimens (RI-CBT) for adults. We reviewed medical records of 140 patients who underwent RI-CBT at Toranomon Hospital between January 2002 and March 2005. All the patients were monitored for CMV-antigenemia weekly, and, if turned positive, received preemptive foscarnet or ganciclovir. Seventy-seven patients developed positive antigenemia at a median onset of day 35 (range, 4-92) after transplant. Median of the maximal number of CMV pp65-positive cells per 50,000 cells was 22 (range, 1-1806). CMV disease developed in 22 patients on a median of day 35 (range, 15-106); 21 had enterocolitis and 1 had adrenalitis. CMV antigenemia had not been detected in 2 patients, when CMV disease was diagnosed. CMV disease was successfully treated using ganciclovir or foscarnet in 14 patients. The other 8 patients died without improvement of CMV disease. In multivariate analysis, grade II-IV acute graft-versus-host disease was a risk factor of CMV disease (relative risk 3.48, 95% confidential interval 1.47-8.23). CMV reactivation and disease develop early after RI-CBT. CMV enterocolitis may be a common complication after RI-CBT.     

GRO-alpha in Human Serum: Differences Related to Age and Sex

PROBLEM: Human GRO-alpha (GRO-α) is a new member of the chemokine family that is supposed to play an important role in inflammatory and immune reactions. We established a sandwich enzyme-linked immunoassay (ELISA) system with polyclonal antibodies against human GRO-α and investigated the serum level of healthy donors to establish normal ranges for this chemokine in adults. METHODS: GRO-α concentrations were measured cross-sectionally in the sera of 240 healthy adults. The variability of serum GRO-α levels was also measured in normal volunteers, samples from whom were obtained by sequential venipunctures or by a small plastic cannula with a heparin-saline lock, to determine short-term variability. RESULTS: Whereas there was no difference between the concentration of human GRO-α from men (logarithmic mean, 77.6 pg/ml, n = 120) and that from women with normal menstrual cycles (log mean, 71.6 pg/ml, n = 73), the concentration from postmenopausal women (log mean 45.0 pg/ml, n = 31) was lower than that from women with normal menstrual cycles (log mean 71.6 pg/ml, n = 73). However, we could not detect any significant difference between healthy donors' serum levels and those of donors with acute inflammation. Fewer variations were recognized in the case of the sequential venipunctures method than in that of the heparin-saline lock method. CONCLUSION: We found that the GRO-α concentration of postmenopausal women was significantly lower than that of women with normal menstrual cycles. These results suggest the GRO-α serum levels of normal healthy women may have some correlation with sex hormones.     

Congestive heart failure due to a combination of atrial fibrillation and tricuspid regurgitation: clinical course and echocardiographical analysis

The combination of atrial fibrillation (Af) and tricuspid regurgitation (TR) may induce the congestive heart failure (HF) due to insufficient output. However, these associations have not been elucidated. We examined the clinical course with echocardiographic data in 119 patients with Af and/or TR and/or HF. Their mean age was 65 +/- 14 years. The patients were classified into 6 groups as follows: Group I contained patients with Af, TR and HF(n = 13); Group II contained patients with Af and TR, without HF(n = 59); Group III contained patients with TR and HF, without Af(n = 15); Group IV contained patients with TR and without AF and HF(n = 15); Group V contained patients with Af and HF, without TR(n = 24); Group VI contained patients with Af and without TR and HF(n = 13). The fractional shortening and ejection fraction in Group I and II were significantly (p < 0.05) decreased compared to Group IV. Moreover, the fractional shortening in Group V was significantly (p < 0.05) lower than in Group IV. Among 72 cases in Group I and II, 24 cases were followed by echocardiography for 2 to 5 years. Eight cases of HF exhibited a no HF state and 1 demonstrated had the HF state. All 9 cases (38%) showed a decreased heart rate and no alternation of TR. Four of the 24 patients (17%) had developed worsened TR and a lowered ejection fraction but none developed them became HF. Therefore, tachycardia may be an accelerative factor for HF due to the combination of Af and TR, not due to the grade of TR.     

Epidermoid Cyst Derived from an Accessory Spleen in the Pancreas

A rare case of splenic epidermoid cyst (SEC) of the pancreas discovered in a 32-year-old Japanese female is reported. The lesion, 5x6cm in size including caseous material and serous fluid in the lumen, was discovered by ultrasonography and computed tomography at the tail of the pancreas and was easily removed. Histopathologically, the cystic wall consisted of three components: the inside was lined by mature squamous epithelium with keratinization, the middle layer consisted of splenic pulp with a sinus structure, and the peripheral layer was dense fibrous connective tissue in which some involutional pancreatic ducts and islets were recognized. The literature about SEC of the pancreas is discussed in comparison with other types of epidermoid cyst including lymphoepithelial cyst and dermoid cyst in the pancreas. Acta Pathol Jpn 41: 916 921, 1991.     

Survival of patients with HTLV-I-associated lymph node lesions

Adult T-cell leukaemia/lymphoma (ATLL), is a malignant condition associated with human T-cell leukaemia virus type I (HTLV-I). Usually, although not uniformly, histopathological examination of the lymph nodes shows a pleomorphic type. In addition, some patients with pre-overt ATLL show a Hodgkin's disease-like morphology and lymph nodes in non-neoplastic carriers show features of lymphadenitis. To characterize further the clinicopathological features of HTLV-I-associated lymphadenopathy, the histopathological features of the lymph nodes of 289 patients were classified into five types: lymphadenitis ( n=14), Hodgkin's-like (Hodgkin's) ( n=18), pleomorphic (medium and large cells) ( n=219), pleomorphic small cell ( n=11), and anaplastic large cell (ALC) ( n=27). Survival data were analysed according to the histopathological features of the lymph nodes. The pleomorphic type, which showed typical features of ATLL, was associated with a highly aggressive course and an initially high mortality, followed by a rapid decrease in survival. This pattern was also observed in patients with the ALC type. All cases with lymphadenitis were still alive at the end of the study, while survival progressively decreased in the Hodgkin's type. The small cell type showed an initial rapid decrease in survival followed by a plateau. These results show that the survival trends of patients with pleomorphic and anaplastic lymph node lesions are similar to those with ATL lymphoma, while patients with the lymphadenitis type of lesion were considered to have a non-neoplatic status. There is at present no effective therapy for ATLL, but in the future, these classification and survival data might be useful for the selection of appropriate chemotherapeutic regimens for patients with ATLL.