Myeloid leukemoid reaction after initial azacitidine therapy for chronic myelomonocytic leukemia.

The development of myeloid leukocytosis in leukemia patients during antileukemic treatment requires a differential diagnosis between myeloid leukemoid reaction and leukemia progression. We herein report the case of an 80-year-old Japanese man with chronic myelomonocytic leukemia (CMML) who developed marked myeloid leukocytosis (36.3 × 109/L) with 32.5% monocytes and 48% neutrophils about 4 weeks after the initial 5-azacitidine (AZA) treatment. The leukocytosis was unlikely to be attributed to infection and adverse drug reaction. As it resolved in a few days without any interventions, the transient myeloid leukocytosis was confirmed to be a myeloid leukemoid reaction. After four cycles of AZA treatment, leukemic blasts in the bone marrow decreased and the patient became transfusion-independent. Interestingly, levels of serum G-CSF showed a similar trend to the myeloid leukocytosis, while those of serum GM-CSF and IL-17 were undetectable throughout the clinical course, suggesting that a differentiation response to AZA treatment might lead to the myeloid leukemoid reaction. Our case implies that a marked but transient myeloid leukemoid reaction mimicking CMML progression can develop during AZA treatment, which requires careful clinical monitoring and differential diagnosis.

[Multiple extramedullary plasmacytomas responding to a reduced dose of carfilzomib following drug-induced thrombotic microangiopathy].

Herein, we report the findings of a 79-year-old male patient who presented with multiple extramedullary plasmacytomas following a relapse of primary plasma cell leukemia. He developed thrombotic microangiopathy (TMA) while receiving carfilzomib, lenalidomide, and dexamethasone (KLd) therapy. He was diagnosed with plasma cell leukemia 3 years ago; he demonstrated a very good partial response (VGPR) after undergoing two regimens, including either bortezomib or lenalidomide, and he had been followed up without any other treatment due to complications of infection. Following relapse, KLd was initiated. On day 7 of KLd, TMA developed; therefore, the treatment was discontinued. The TMA improved only with the discontinuation of KLd. A reduced dose of KLd was readministered; the TMA did not relapse. He demonstrated VGPR after three courses of reduced-KLd; he has since remained in remission through ten courses. Therefore, carfilzomib therapy may be useful in relapsing and refractory cases. Drug-induced TMA has been reported to be caused by either immune-mediated or dose-dependent toxicity mechanisms. In patients who develop dose-dependent TMA with carfilzomib, dose reduction could be considered in cases showing an effective response to the treatment.

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.

[Isolation of Neisseria elongata subsp. elongata Isolated from an Acute Myelogenous Leukemia Patient].

Gram-negative cocci with a rod-like shape were isolated from a blood sample of a patient with acute myelogenous leukemia (AML). The 16S rRNA sequence of the isolate was similar to that of Neisseria elongata. Because previous reports about N. elongata as a pathogen have been extremely rare, more reliable identification seemed to be needed. We thus additionally performed a Multilocus Sequencing Analysis (MLSA) based on another four regions (argF, rho, recA, glnA), and confirmed the identification of N. elongata. The results from the MLSA identified the species; however, we could not identify the isolates into subspecies from the sequences. Three subspecies of N. elongata (N. elongata subsp. elongata, N. elongata subsp. glycolytica and N. elongata subsp. nitroreducens) were classified based on three definitive characteristics (catalase possession, nitrite reducibility, and acid from glucose). The results of the tests of three characteristics supported the identification of the isolate as N. elongata subsp. elongata. Therefore we determined the isolate from the AML patient to be N. elongata subsp. elongata.

Varicella infection complicated by marked thrombocytopenia.

We report a rare case of adult varicella complicated by marked thrombocytopenia. A 49-year-old woman presented with fever and rash for 3 days. Blood examination revealed marked thrombocytopenia (2.7 × 10(4)/µL). Varicella infection was diagnosed after elevated levels of varicella zoster virus IgM and IgG antibodies were observed 2 weeks later. In this case, thrombocytopenia was due to varicella infection, and the mechanism was estimated to be non-immunological. Because varicella infection complicated by thrombocytopenia may result in fatal bleeding, thrombocytopenia in patients with varicella warrants close attention.

Molecular confirmation of transmission route of Staphylococcus intermedius in mastoid cavity infection from dog saliva.

We report a case of infection of a mastoid cavity after mastoidectomy had been performed for chronic otitis media with cholesteatoma. The infection was caused by Staphylococcus intermedius after a pet dog had licked the patient's ears. Bacterial strains from the dog's saliva and the otorrhea in the patient were confirmed to be identical by pulsed-field gel electrophoresis analysis. The possibility of an oral transmission route of S. intermedius from pets to humans should be noted.

[Massive hemolysis following allogeneic peripheral blood stem cell transplantation with minor ABO incompatibility].

We observed massive hemolysis after allogeneic peripheral blood stem cell transplantation with minor ABO incompatibility (the donor was group O and the recipient group A). The patient was a 21-year-old man diagnosed as having Philadelphia chromosome-positive chronic myelogenous leukemia. On day 7 post-transplant, there was abrupt onset of massive hemolysis necessitating supportive treatment with transfusions. During the allogeneic peripheral blood stem cell transplantation the patient had received 2 x 10(8) CD3 positive cells/kg and 7.8 x 10(7) CD19 positive cells/kg donor lymphocytes with stem cells. The present case shows that in allogeneic peripheral blood stem cell transplantation with ABO incompatibility, severe hemolysis occurs early after transplantation presumably due to the transfusion of a large number of lymphocytes.

[Hospital outbreak of viral gastroenteritis attributed to Norwalk-like viruses in Japan].

BACKGROUND: Norwalk-like viruses (NLVs), RNA viruses in the family of Caliciviridae, are known as a pathogen of nonbacterial food-borne gastroenteritis associated with eating raw oysters. NLV can spread from person to person with strong infectivity and can cause large epidemics in communities, schools, nursing homes, and hospitals. Here, we describe an outbreak of gastroenteritis associated with NLVs, possibly introduced from outside the hospital, in four different wards on four different occasions between November 1999 and April 2000, in a university affiliated hospital in Tokyo. METHOD: Total 61 specimens (stool or vomitus) from 46 patients and staffs were collected. Reverse transcription-polymerase chain reaction (RT-PCR) test was performed on each specimen. Standard precautions were applied strictly as control. RESULT: NLV was detected in 40 specimens from 28 patients and staff (20 patients, 8 nurses). Attack rate of the patients were 0.19, nurses were 0.15, and doctors were 0.07. CONCLUSION: The spread of NLV from person to person is considered to be a major infection pathway in each ward. A nurse is at a high risk of infection as a patient in some wards. A stringent policy of control must be applied.