A Case of Refractory Langerhans Cell Histiocytosis Complicated with Hemophagocytic Lymphohistiocytosis Rescued by Cord Blood Transplantation with Reduced-intensity Conditioning

　We diagnosed a female infant with Langerhans cell histiocytosis (LCH) who was refractory to conventional chemotherapy. She showed refractory inflammation that was complicated with hemophagocytic lymphohistiocytosis (HLH) during LCH chemotherapy; therefore, we changed the protocol to HLH2004 (dexamethasone, cyclosporine A and VP16). However, there were no signs of hematological recovery. We therefore performed cord blood transplantation with reduced-intensity conditioning, and she achieved complete remission for over 2 years. As salvage therapy for refractory LCH, hematopoietic stem cell transplantation may be a good therapeutic choice, especially when LCH is complicated with HLH

A Long-term Survivor after Congenital Acute Myeloid Leukemia with t(8 ; 16)(p11 ; p13)

The treatment of patients with congenital leukemia is difficult and often results in a poor prognosis. We present here the case of a female child with congenital acute myeloid leukemia (AML) with t(8 ; 16) (p11 ; p13) who received chemotherapy and survived for more than 10 years without relapse. A novel MOZ-CBP chimera was found in her diagnostic sample. Although adult AML patients with MOZ-CBP have mainly been reported as having therapy-related AML and showed poor prognoses, the present case supports the idea that AML with MOZ-CBP in the pediatric population might show better prognoses

Two Relapsed Stage III Childhood Anaplastic Large Cell Lymphoma Patients with NPM-ALK Fusion in Bone Marrow from Initial Diagnosis

Childhood anaplastic large cell lymphoma (ALCL) accounts for approx. 10–30 of cases of non-Hodgkin lymphoma, and the ALCL99 study reported 60–75 disease-free survival; however, a relatively high relapse rate was observed (25–30 ). We report 2 patients with Stage III ALCL who relapsed 6–18 months after the end of ALCL99 chemotherapy. A retrospective molecular analysis identified the nucleophosmin (NPM)-anaplastic lymphoma kinase (ALK) fusion gene in the first diagnostic bone marrow samples taken from both patients. However, antibodies against the ALK protein appeared to be relatively low in the serum of both patients (×100 and ×750). An increase in chemotherapy intensity may be beneficial if Stage III ALCL patients are shown to be NPM-ALK chimera-positive in the first diagnostic bone marrow sample

Two Relapsed Stage III Childhood Anaplastic Large Cell Lymphoma Patients with NPM-ALK Fusion in Bone Marrow from Initial Diagnosis

Childhood anaplastic large cell lymphoma (ALCL) accounts for approx. 10–30 of cases of non-Hodgkin lymphoma, and the ALCL99 study reported 60–75 disease-free survival; however, a relatively high relapse rate was observed (25–30 ). We report 2 patients with Stage III ALCL who relapsed 6–18 months after the end of ALCL99 chemotherapy. A retrospective molecular analysis identified the nucleophosmin (NPM)-anaplastic lymphoma kinase (ALK) fusion gene in the first diagnostic bone marrow samples taken from both patients. However, antibodies against the ALK protein appeared to be relatively low in the serum of both patients (×100 and ×750). An increase in chemotherapy intensity may be beneficial if Stage III ALCL patients are shown to be NPM-ALK chimera-positive in the first diagnostic bone marrow sample

Clozapine and Antipsychotic Monotherapy

Background: Although clozapine is effective for treatment-resistant schizophrenia (TRS), the rate of clozapine prescription is still low. Whereas antipsychotic monotherapy is recommended in clinical practice guidelines, the rate of antipsychotic polypharmacy is still high. There is little evidence on whether a clozapine prescription influences changes in the rate of monotherapy and polypharmacy, including antipsychotics and other psychotropics. We therefore hypothesized that the rate of antipsychotic monotherapy in patients with TRS who were prescribed clozapine would be higher than that in patients with schizophrenia who were not prescribed clozapine. Methods: We assessed 8306 patients with schizophrenia nationwide from 178 institutions in Japan from 2016 to 2019. We analyzed the psychotropic prescription data at discharge in patients diagnosed with TRS and with no description of TRS (ND-TRS) based on the diagnosis listed in the discharge summary. Results: The rate of antipsychotic monotherapy in the TRS with clozapine group (91.3%) was significantly higher than that in the TRS without clozapine group (45.9%; P < 2.0 × 10−16) and the ND-TRS without clozapine group (54.7%; P < 2.0 × 10−16). The rate of antipsychotic monotherapy without any other concomitant psychotropics in the TRS with clozapine group (26.5%) was significantly higher than that in the TRS without clozapine group (12.6%; P = 1.1 × 10−6) and the ND-TRS without clozapine group (17.0%; P = 5.9 × 10−6). Conclusions: Clozapine prescription could be associated with a high rate of antipsychotic monotherapy. Patients will benefit from the correct diagnosis of TRS and thus from proper clozapine prescription

Subjective assessment of participants in education programs on clinical practice guidelines in the field of psychiatry

The Effectiveness of Guidelines for Dissemination and Education in psychiatric treatment (EGUIDE) project, which is a nationwide dissemination and implementation program for clinical practice guidelines (CPGs) in the field of psychiatry, is currently ongoing. In the current study, a subjective assessment of the participants in the EGUIDE programs was assessed using a questionnaire. Then, the relationships between the subjective assessment, the characteristics of the participants, and the clinical knowledge of the CPGs were evaluated. More than 90% of the participants gave a high rating for the components of content, recommendation, knowledge, skill, and adherence, but not for the component of confidence. A positive correlation was found between years of professional experience and the score of confidence. These results suggest that it may be necessary to apply the knowledge and skills of CPGs obtained in the education programs into practice to increase confidence in the proper use of psychiatric therapies based on CPGs

The Relationship between Individual Difference in Repressive Stles and Grief Reaction Coping Behaviors, and Onset of Pathological Grief, Anxiety and Mood Disorders after Child Loss

We examined the relationship between parental repressive styles, grief reaction/post-bereavement coping behavior and psychiatric symptoms in parents who had lost young children, In Study I, we administered a set of questionnaires to a sample of 177 parents who had experienced bereavement of children within the past several years in order to inquire about grief response, coping behavior, repressive styles, and social support .After controlling for sex, age, income, and self esteem, 1) subjects who had high anxiety (sensitizer and repressive-anxiety) showed stronger grief response whereas those with low anxiety (repressor and low-anxiety) showed weak response; 2) repressors were more likely to accept death and resolute grief after the loss than others; 3) those who had higher anxiety were more likely to ruminate after death; and 4) sensitizers were less likely to satisfy perceived social support. In Study II we interviewed 60 bereaved parents using a semi-structured diagnostic interview schedule to yield diagnoses of the parents based on the Diagnostic and Statistical Manual of Mental Disorders 4th Edition (DSM-IV). Parents with repressive-anxiety were more likely to manifest pathological grief and those parents recognized as sensitizers were more likely to have an onset of DSM-IV Major Depression or Social Phobia after the child\u27s death.抑圧様式と悲嘆反応、死別後の対処行動、精神症状との関連性について検討した。研究Iでは幼い子どもと死別した親177名を対象に悲嘆反応、対処行動、抑圧様式、ソーシャルサポートを測定した。性別、年齢、収入などを統制し分析した結果、①不安水準の高い sensitizer, repressive Anxiety 各群では悲嘆反応が強心不安水準の低い repressor, low-anxiety各群では悲嘆反応が弱い、②repressor群では悲嘆がすでに解決したことと認知し、死別体験を肯定的に捉える傾向が強い、③不安水準が高いと死別後に内的に考え込む対処を多く行う、④sensitizer群では知覚されたソーシャルサポートに対する満足度が低い傾向にあった。研究IIでは60名に半構造化面接を行い、死別後に罹患した精神疾患の診断を行った。その結果，⑤病的悲嘆はrepressive- anxiety傾向と，⑥大うつ病性障害や社会恐怖はsensitization傾向とそれぞれ関連することが示された

The Measurement of Grief and Coping after Loss of a Child

We administered Grief Response Scale (GRS), Japanese translation of "Core Bereavement Items," and a set of newly developed coping　behavior items (Scale for Coping with　Bereavement; SCB) to 48 fathers and 127　mothers who experienced the loss of a child within several years. Although a confirmatory factor analysis of GRS did not support the original seven-factor model byBurnett et al., an exploratory factor analysis yielded four factors: lmage and sorrow, Sense of presence， Non-resolution and conflict, and Resolution of grief. Grief reaction was significantly greater in mothers than in fathers. An exploratory factor analysis of SCB produced five factors: Distraction, Ruminative response, A cceptance and overcome, Support-seeking behavior, and Religious activity and existential meaning. As compared to fathers, mothers ruminated, sought support from others, and had religious activity more frequently. These results suggest that different aspects of grief were related with coping with bereavement after controlling for age, sex, income, and level of manifest anxiety