Does time of CCI measurement affect the evaluation of platelet transfusion effectiveness?

The measurement of corrected count increment at 1-h post-transfusion (CCI-1 h) of platelet concentrate (PC) transfusion is recommended, but in the revised Japanese Guideline (2017) it was changed to "after 10-min to 1-h", following the revision of the guidelines from Western countries. Here, we aimed to investigate on the feasibility to apply the CCI measured at 10-min or 30-min post-transfusion as the surrogate of CCI-1 h. Peripheral blood was collected at 10-min, 30-min and 1-h post-transfusion of PC and the effectiveness of the transfusion was analyzed based on the CCI. In the period from December 2017 to February 2020, 8 patients, who received multiple PC transfusion (total 208) at our institution, were analyzed. We performed the univariate analyses to examine the relationship between CCI value and the categorical variables, p-value <0.1 was obtained for gender (p = 2.91 × 10-19), fever after transfusion (p = 0.0163). The qualitative variables, namely measurement time (p = 0.0553), also showed p-value <0.1. Using these factors as covariates in the mixed effect model, we found that the measurement time (p = 0.0007) had a significant effect on the CCI value when looking at fixed effects. Although there is a tendency for decreased CCI values with time progression, the slope of the change in the mixed model was -0.00307, indicating that the CCI difference among the 3 measurements was small. Here we provide evidence that CCI measured at 10-min and 30-min post-transfusion give results comparable to those measured at 1-h post-transfusion, under the Japanese practice of platelet transfusion, which relies on 100 % single-donor apheresis PC, and ABO-identical whenever possible.

Azacitidine-associated haemorrhagic enteritis in a case of myelodysplastic syndrome: A case report.

WHAT IS KNOWN AND OBJECTIVE: 5-Azacitidine (AZA) is an agent widely used to treat myelodysplastic syndrome (MDS). CASE DESCRIPTION: We herein report an 83-year-old woman diagnosed with MDS who was treated with AZA. She tolerated the first cycle of AZA; however, severe adverse events involving haemorrhagic enteritis with multiple intestinal ulcers developed after the second and third cycles. Additionally, the interval between the administration of AZA and the development of haematochezia shortened with each cycle of AZA. WHAT IS NEW AND CONCLUSION: We herein report as-yet-undescribed potential side effects, AZA-associated haemorrhagic enteritis that should be kept in mind.

Multiple HLA-matched platelet transfusions for a single patient with broad anti-HLA antibodies: a case report.

The efficacy of 30 platelet concentrate (PC) products transfused to a patient with myelodysplastic syndrome (MDS) was evaluated by calculating the 1-hour post-transfusion corrected count increment (1h-CCI). Of the 30 transfusions, all HLA-A/B-matched, the cross-match (CM) test was negative in 23 (CM(-)-PC) and weakly positive (CM(+)-PC) in 2, and the CM test was not conducted in 5 (non-CM-PC). The effective rate was higher with CM(-)-PC compared to non-CM-PC (82.6% vs 60%), but statistical significance was not achieved, which suggested that the CM test of PC may still be a not satisfactorily effective predictor of PC refractoriness. Studies are ongoing in Japan to confirm on the importance of CM test of PC.

[Case of renal parenchymal malakoplakia presenting as sepsis and treated with nephrectomy].

Malakoplakia is a rare chronic inflammatory condition characterized by defective macrophage function, most of which involve the genitourinary tract, and renal parenchymal involvement is uncommon. We present a case of malakoplakia affecting renal parenchyma. A 46-year-old woman with pyrexia and jaundice was referred to our department. Abdominal enhanced CT scan revealed a left pyelonephritis with ureteral stone and bilateral renal abscesses. Despite the insertion of a left ureteral stent and administration of antibiotics, the patient showed persistent high fever and elevated CRP, and no obvious improvement in clinical and imaging data. In view of the limited effectiveness of the conservative treatment in this case, we decided to perform left nephrectomy. The diagnosis of malakoplakia was made based on the histopathological findings of von Hansemann cells and Michaelis-Guttmann bodies detected in the nephrectomy specimen. She is clinically healthy up to the present (50 months after surgery) with normal clinical indicators and CT findings.

Brachial-brachial autogenous arteriovenous fistula in a dialysis patient with Staphylococcus aureus bacteremia.

As the number of patients on hemodialysis increases, there will also be an increase in the number of patients with inadequate superficial veins for the creation of an autogenous arteriovenous fistula (AVF). In those patients, medical devices such as vascular prostheses or tunneled-cuffed catheters are necessary to maintain dialysis access. However, these devices are frequently associated with bacterial infection. We recently encountered a dialysis patient who underwent tunneled-cuffed catheter insertion because of the lack of usable superficial veins for autogenous access, and this patient subsequently developed catheter-related Staphylococcus aureus bacteremia with multiple metastatic infections. Despite immediate removal of the catheter, the infection persisted over an extended period, which was a condition precluding the further use of catheters or other prosthetic materials. To handle this situation, we utilized the deep brachial vein to construct an autogenous AVF. After ligating numerous branches, the vein was anastomosed to the brachial artery and then transposed to the subcutaneous space. The newly constructed autogenous AVF, which successfully kept the patient free from foreign materials, greatly contributed to the relief of persistent infection. Although the brachial vein is rarely used for AVF creation, we suggest that it can serve as an option to create an alternative AVF in a patient with inadequate superficial veins.

[Analysis of multiple cancers in autopsy cases of elderly hematological malignancies].

To investigate the actual situation of multiple cancers including hematological malignancies, 266 autopsy cases with one or more hematological malignancies were compiled from autopsy case files between January 1995 and October 2006 in our hospital. The median age at death was 75 years (range 48 to 102 yr). Of 266 cases, 72 (27.1%) had multiple cancers. Of these 72 cases, 62 cases were complicated with non-hematological malignancy, and 10 cases showed duplication of other hematological malignancies. Prostate and colon cancers were frequent as complicating cancers. Seventeen of 256 cases without duplication of other hematological malignancies demonstrated 3 or 4 cancers (6.6%). Of 10 cases showing duplication of other hematological malignancies, 9 cases had NHL. The rate of multiple cancers in elderly patients with hematological malignancy was higher than that of non-hematological cancers.

Abnormal N-glycosylation of the immunoglobulin G kappa chain in a multiple myeloma patient with crystalglobulinemia: case report.

Spontaneous crystallization of monoclonal immunoglobulins (crystalglobulin) is a rare complication of multiple myeloma. We describe a 64-year-old Japanese man with skin ulcers and renal failure associated with immunoglobulin G kappa multiple myeloma. Crystallized immunoglobulin was detected in his serum at room temperature. Analysis of the patient's crystalglobulin by sodium dodecyl sulfate-polyacrylamide gel electrophoresis and mass spectrometry suggested that the crystallization was due to abnormal glycosylation of the immunoglobulin light chain. Treatment with thalidomide and dexamethasone improved the severe skin ulcers on the patient's extremities and partially reversed his renal failure. This report is the first of abnormal glycosylation of immunoglobulin possibly caused by modification of N-glycans in the light chain. We concluded that abnormal glycosylation of the immunoglobulin light chain might be the cause of the patient's skin ulcers and renal dysfunction.

[Mantle cell lymphoma markedly infiltrated into adrenal glands with adrenal insufficiency].

A 66-year-old male was admitted to our hospital complaining of bilateral hypochondrial pain, back pain and loss of weight in May, 2002. Superficial lymph nodes were not palpable on admission. The leukocyte count was 3430/microl, hemoglobin concentration, 13.0g/dl, and platelet count, 174000/microl. LDH, soluble IL-2 receptor, ACTH and cortisol values were out of the normal range (LDH 1368IU/l, sIL-2R 2630U/ml, ACTH 132pg/ml, cortisol 7.4microg/dl). Abdominal CT scan showed bilateral adrenal masses, and abnormal uptake of Ga-scintigraphy was seen correspondent with the bilateral adrenal masses. The histological diagnosis of bilateral adrenal masses cannot be performed because of the bleeding tendency, but atypical cells were observed in the patient's bone marrow aspirate. Surface marker analysis of atypical cells showed CD5+, cyclin D1+, CD19+, CD20+ and HLA-DR+. From these results we diagnosed this case as a mantle cell lymphoma (stage IV B) markedly infiltrated into the adrenal glands with adrenal insufficiency. The bilateral adrenal masses dramatically reduced in size after CHOP chemotherapy with hydrocortisone supplementation. We report on the present case and summarize the reports of adrenal grand-infiltrating lymphomas.

VHL tumor suppressor gene: its mutation and protein level in renal cell carcinoma.

In this study, we identified mutations in the von Hippel-Lindau gene (VHL) in patients with sporadic clear cell renal carcinoma and analyzed the relationship between these VHL mutations and VHL protein (pVHL) expression levels. Analysis of VHL coding region mutations by direct polymerase chain reaction in 39 patients with clear cell renal carcinoma, who had all undergone radical nephrectomy, revealed heterozygous mutations in 7 (17.9%) of the 39 patients, including 2 novel mutations. Analysis of the levels of pVHL expression in tumor tissue samples from the 7 patients with mutations by Western blot analysis demonstrated that pVHL was not expressed in 3 patients, and that 3 subjects expressed about 50%, and 1 was similar to the normal level. These results suggest that the 3 patients who did not express pVHL had both mutation and loss of heterozygosity, and that the 3 patients who expressed about 50% of the normal pVHL level had heterozygous mutations.

Prognosis of elderly patients with acute myelogenous leukemia: analysis of 126 AML cases.

We retrospectively analyzed 126 acute myelogenous leukemia (AML) patients aged > or =60 years who had all been referred to the same hematological department between 1989 and 1999. In 76 de novo AML cases, 53 patients (median age, 72 years) were treated with combination chemotherapy (CT) for remission induction. Complete remission (CR) rate was 57.1%. The median overall survival (OS) was 16 months, and the rate of 3-year OS was 28%. The favorable prognostic factors were performance status < or =2, cholinesterase > or =100 IU, and intermediate or favorable karyotype (P < .01). Seventeen patients (median age, 78 years) with hypocellular bone marrow or poor general condition were treated with low-dose cytosine arabinoside (LDAraC). In these patients, the CR rate was 50% and the median OS was 11 months, with an OS estimate at 3 years of 14%. All patients with hypocellular bone marrow who received LDAraC for 21 days achieved CR. In 50 patients who developed AML following a myelodysplastic syndrome (MDS/AML), 22 patients (median age, 74 years) were treated with CT, and 14 (median age, 74 years) patients were treated with LDAraC. The CR rates were 22.7% and 21.4%, respectively, and the median OS durations were 8 months and 11 months, respectively. There were no significant factors that would indicate a good prognosis in MDS/AML patients.

[Bone lesions in elderly multiple myeloma].

We investigated the incidence of bone lesions in elderly cases of multiple myeloma (MM) and the course of those lesions, and also evaluated the relationships of skeletal symptoms with prognostic factors, and prognosis. The subjects were 146 patients, aged 65 years or more (median age 74, range 65-97 year), who were admitted to 11 institutions between January, 1988 and December, 1997. They consisted of 64 men and 82 women. The disease type was IgG type in 88 patients, IgA type in 37 patients, Bence-Jones (BJ) type in 17 patients, IgD type in three patients, and non-secretory type in one patient. Bone lesions in elderly MM patients were compared with those in 65 non-elderly MM patients. Skeletal symptoms were noted in 104 patients, and bone pain in 75 patients at the time of diagnosis. The bone lesions were evaluated as only osteolytic lesions in 26 patients, osteolytic lesions + osteoporosis in 23 patients, only osteoporosis in 2 patients and pathologic bone fractures in 53 patients. The occurrence rate of osteoporosis plus osteolytic lesion was higher in elderly patients (63.5%) than that in non-elderly patients (NE-MM group) (28.3%) (p < 0.0001). The bone lesions were most often observed in lumbar vertebrae (58.7%), cranial bone (56.7%), thoracic vertebrae (40.4%) and ribs (27.9%). The occurrence rate of bone lesion in lumbar vertebrae was higher in elderly patients (58.7%) than that in non-elderly patients (22.6%) (p < 0.0001). The life activities were limited in 71 patients because of the bone lesions. The relationship between the prognostic factors of MM and bone lesions was evaluated. There was a significant difference in the serum Ca level between patients with and without bone pain (P < 0.0001) and between those with and without pathologic bone fracture (P < 0.01). There was a significant difference in the appearance rate of plasma cells in the bone marrow between the patients with and without bone lesions (P < 0.05), between those with and without bone pain (P < 0.01), and between those with and without pathologic fracture (P < 0.05). There was a significant difference in the serum beta 2-microglobulin level between the patients with and without bone pain, and between those with and without pathologic fracture. There were no significant differences in survival times between elderly MM patients with and without bone lesions, bone pain and pathological bone fractures, while significant differences of survival times were found between non-elderly MM patients with and without bone lesions, bone pain and pathological bone fractures (P < 0.05, each). These data suggest that there are some differences in bone lesions between elderly and non-elderly MM patients.