RESUMO
BACKGROUND: New-onset allergic diseases, such as food allergy or atopic dermatitis, can develop after allogeneic transplantation. There are limited reports of new-onset atopic dermatitis after allogeneic hematopoietic stem cell transplantation in children and adolescents, and its treatment is yet to be established. The pathogenesis may differ from typical atopic dermatitis in terms of alloimmunity including graft-versus-host disease. METHODS: We present five children and adolescents with new-onset atopic dermatitis after allogeneic hematopoietic stem cell transplantation. The characteristics and clinical profiles of skin treatment after hematopoietic stem cell transplantation are summarized. RESULTS: Graft-versus-host disease prophylaxis included systemic tacrolimus for all patients. After hematopoietic stem cell transplantation, all patients achieved complete donor chimerism of the bone marrow and had acute graft-versus-host disease of the skin. After engraftment, all patients had skin lesions that met the international consensus diagnostic criteria for atopic dermatitis. None of the patients met the diagnostic criteria for chronic graft-versus-host disease. Topical therapy and skin care based on atopic dermatitis guidelines improved skin condition and atopic dermatitis severity scores in all patients. In addition, type 2 inflammatory markers improved accordingly. CONCLUSION: Topical therapy and skin care may be effective for transplant-related atopic dermatitis after hematopoietic stem cell transplantation. When extensive dermatitis is observed after hematopoietic stem cell transplantation, this treatment may avoid excessive immunosuppressive therapy if it meets the diagnostic criteria for atopic dermatitis.
Assuntos
Dermatite Atópica , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Criança , Humanos , Adolescente , Dermatite Atópica/terapia , Dermatite Atópica/complicações , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/prevenção & controle , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante Homólogo/efeitos adversos , Higiene da Pele/efeitos adversos , Condicionamento Pré-Transplante/efeitos adversosRESUMO
Mutations in the MECOM encoding EVI1 are observed in infants who have radioulnar synostosis with amegakaryocytic thrombocytopenia. MECOM-associated syndrome was proposed based on clinical heterogeneity. Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative treatment for progressive bone marrow failure. However, data regarding allogeneic HSCT for this rare disease are limited. We retrospectively assessed overall survival, conditioning regimen, regimen-related toxicities and long-term sequelae in six patients treated with allogeneic HSCT. All patients received a reduced-intensity conditioning (RIC) regimen consisting of fludarabine, cyclophosphamide or melphalan, and rabbit anti-thymocyte globulin and/or low-dose total body/thoracic-abdominal/total lymphoid irradiation, followed by allogeneic bone marrow or cord blood transplantation from unrelated donors between 4 and 18 months of age. All patients survived and achieved stable engraftment and complete chimerization with the donor type. Moreover, no patient experienced severe regimen-related toxicities, and only lower grades of acute graft-versus-host disease were observed. Three patients treated with low-dose irradiation had relatively short stature compared to three patients not treated with irradiation. Therefore, allogeneic HSCT with RIC is an effective and feasible treatment for infants with MECOM-associated syndrome. Future studies are needed to evaluate the use of low-dose irradiation to avoid risks of other long-term sequelae.
Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Humanos , Estudos Retrospectivos , Transplante Homólogo , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Medula Óssea , Fatores de Transcrição , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/prevenção & controle , Doença Enxerto-Hospedeiro/tratamento farmacológico , Doadores não Relacionados , Condicionamento Pré-Transplante , Vidarabina/uso terapêutico , Proteína do Locus do Complexo MDS1 e EVI1RESUMO
BACKGROUND: Better therapeutic options other than conventional chemotherapy for pediatric patients with refractory Langerhans cell histiocytosis (LCH) remain undetermined. CASE: We successfully treated two patients with refractory and risk organ negative LCH with clofarabine (CLO) monotherapy after recurrence. We administered total 23 courses of CLO monotherapy in patient 1 and 4 courses in patient 2. Both patients had distinct clinical manifestations but achieved a durable complete response with acceptable adverse effects of transient myelosuppression. CLO monotherapy was still effective when he had the second recurrent lesion after first completion of CLO in patient 1. We could discontinue prednisolone to control his refractory inflammation of LCH after completing CLO chemotherapy in patient 2. CONCLUSION: Although large-scale studies are warranted, CLO monotherapy could be a therapeutic option for high efficacy and feasibility besides other intensive combination chemotherapies or allogeneic hematopoietic stem cell transplantation for refractory LCH without risk organ involvement in children.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Histiocitose de Células de Langerhans , Criança , Clofarabina/uso terapêutico , Histiocitose de Células de Langerhans/diagnóstico , Histiocitose de Células de Langerhans/tratamento farmacológico , Humanos , Masculino , Indução de RemissãoRESUMO
BACKGROUND: X-linked inhibitor of apoptosis protein (XIAP) deficiency is an infrequent inborn error of immunity that is often associated with refractory inflammatory bowel disease (IBD). The natural course of XIAP deficiency is typically associated with poor prognosis, and hematopoietic cell transplantation (HCT) is the only curative treatment. OBJECTIVE: To study (1) the effect of HCT on patients with XIAP deficiency undergoing HCT, (2) the status of XIAP deficiency-associated IBD after HCT, and (3) the gut microbiota of XIAP deficiency-associated IBD before and after HCT. METHODS: A nationwide survey of patients with XIAP deficiency was conducted. A spreadsheet questionnaire was collected from the physicians. Feces samples collected from the patients before and after HCT and their healthy family members were analyzed. RESULTS: Twenty-six patients with XIAP deficiency underwent HCT by the end of March 2020, and 22 patients (84.6%) survived. All the survivors underwent a fludarabine-based reduced-intensity condition regimen. Acute graft-versus-host disease was observed in 17 patients (65.4%). Nineteen patients experienced refractory IBD before undergoing HCT. IBD improved remarkably after HCT. After HCT, the colonoscopic and pathological symptoms were restored to normal, and the pediatric ulcerative colitis activity index improved significantly. Gut microbiota indicated dysbiosis before HCT; however, it was improved to resemble that of the healthy family members after HCT. CONCLUSIONS: This study revealed that HCT has a favorable outcome for XIAP deficiency. HCT rescues gut inflammation and dysbiosis in patients with XIAP deficiency.
Assuntos
Microbioma Gastrointestinal , Transplante de Células-Tronco Hematopoéticas , Doenças Inflamatórias Intestinais , Disbiose , Doenças Genéticas Ligadas ao Cromossomo X , Humanos , Doenças Inflamatórias Intestinais/terapia , Transtornos Linfoproliferativos , Proteínas Inibidoras de Apoptose Ligadas ao Cromossomo X/genéticaRESUMO
Women are at high risk of hypergonadotropic hypogonadism after hematopoietic cell transplantation (HCT). Hypogonadism is universal after irradiation or busulfan. We hypothesized that reduced intensity conditioning (RIC) might protect ovarian function after HCT. We retrospectively reviewed data from patients with acute leukemia treated according to the Japan Association of Childhood Leukemia Study and nationwide multicenter study protocol. We selected 11 female patients with acute leukemia who received first HCT with RIC, had survived for three or more years after HCT, and were aged ≥ 12 years at the last follow-up visit. Median age at diagnosis, HCT, and last visit were 8, 10, and 17 years. Six patients received HLA-matched bone marrow (BM), two HLA-mismatched BM, and three cord blood. Melphalan was used as conditioning regimen in all patients. At the last visit, six of seven post-pubertal patients at transplantation recovered menstruation, and four of four patients who underwent transplantation at the pre-pubertal began menstruation. Height z scores showed no significant reduction between pre-transplant and post-transplant. No patients received growth hormone treatment. Only one recipient displayed subclinical hypothyroidism. Melphalan-based RIC may be an encouraging option for patients with acute leukemia to avoid ovarian and endocrine dysfunction after HCT.
Assuntos
Preservação da Fertilidade , Transplante de Células-Tronco Hematopoéticas , Leucemia Mieloide Aguda , Melfalan/administração & dosagem , Menstruação , Ovário/fisiopatologia , Leucemia-Linfoma Linfoblástico de Células Precursoras , Condicionamento Pré-Transplante , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Leucemia Mieloide Aguda/fisiopatologia , Leucemia Mieloide Aguda/terapia , Melfalan/efeitos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/fisiopatologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Estudos RetrospectivosRESUMO
BACKGROUND: X-linked inhibitor of apoptosis protein (XIAP) deficiency is a rare immunodeficiency that is characterized by recurrent hemophagocytic lymphohistiocytosis (HLH) and splenomegaly and sometimes associated with refractory inflammatory bowel disease (IBD). Although hematopoietic stem cell transplantation (HSCT) is the only curative therapy, the outcomes of HSCT for XIAP deficiency remain unsatisfactory compared with those for SLAM-associated protein deficiency and familial HLH. AIM: To investigate the outcomes and adverse events of HSCT for patients with XIAP deficiency, a national survey was conducted. METHODS: A spreadsheet questionnaire was sent to physicians who had provided HSCT treatment for patients with XIAP deficiency in Japan. RESULTS: Up to the end of September 2016, 10 patients with XIAP deficiency had undergone HSCT in Japan, 9 of whom (90%) had survived. All surviving patients had received a fludarabine-based reduced intensity conditioning (RIC) regimen. Although 5 patients developed post-HSCT HLH, 4 of them survived after etoposide administration. In addition, the IBD associated with XIAP deficiency improved remarkably after HSCT in all affected cases. CONCLUSION: The RIC regimen and HLH control might be important factors for successful HSCT outcomes, with improved IBD, in patients with XIAP deficiency.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/terapia , Proteínas Inibidoras de Apoptose Ligadas ao Cromossomo X/deficiência , Substituição de Aminoácidos , Códon , Doença Enxerto-Hospedeiro/etiologia , Pesquisas sobre Atenção à Saúde , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/mortalidade , Japão , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/genética , Linfo-Histiocitose Hemofagocítica/mortalidade , Linfo-Histiocitose Hemofagocítica/terapia , Mutação , Análise de Sobrevida , Condicionamento Pré-Transplante , Transplante Homólogo , Resultado do TratamentoRESUMO
Genetic mosaicism for somatic mutations of oncogenes is common in genodermatoses, which can be complicated with extra-cutaneous abnormalities. Here we describe an infant with a congenital anaplastic astrocytoma, a linear syringocystadenoma papilliferum, and ocular abnormalities. The BRAF c.1799T>A p.V600E mutation was detected in both the brain and skin tumor cells but not in the blood or normal skin cells, suggesting somatic mosaicsism for the mutation. Clinically, the brain tumor gradually became life threatening without any response to conventional chemotherapies including carboplatin, etoposide, and temozolomide. Vemurafenib, a BRAF p.V600E inhibitor, was administered daily after the detection of the BRAF mutation. This single-agent therapy was dramatically effective against the anaplastic astrocytoma; the tumor regressed, the cerebrospinal fluid cell count and protein levels decreased to normal levels, and hydrocephalus resolved. Moreover, other lesions including a corneal cyst also responded to vemurafenib. The brain tumor continued shrinking after 6 months of treatment. We present a genodermatosis syndrome associated with BRAF c.1799T>A p.V600E mosaicism. This syndrome may represent a new entity in the mosaic RASopathies, partly overlapping with Schimmelpenning-Feuerstein-Mims syndrome, which is driven by mosaicism of HRAS and/or KRAS activating mutations. Screening for BRAF c.1799T>A p.V600E is especially useful for those with malignant tumors, because it is one of the most-druggable targets.
Assuntos
Adenoma de Glândula Sudorípara/genética , Astrocitoma/genética , Neoplasias Encefálicas/genética , Proteínas Proto-Oncogênicas B-raf/genética , Neoplasias das Glândulas Sudoríparas/genética , Adenoma de Glândula Sudorípara/tratamento farmacológico , Astrocitoma/tratamento farmacológico , Neoplasias Encefálicas/tratamento farmacológico , Olho/patologia , Anormalidades do Olho/genética , Humanos , Indóis/uso terapêutico , Lactente , Mosaicismo , Nevo Sebáceo de Jadassohn/genética , Nascimento Prematuro , Proteínas Proto-Oncogênicas B-raf/antagonistas & inibidores , Sulfonamidas/uso terapêutico , Neoplasias das Glândulas Sudoríparas/tratamento farmacológico , VemurafenibRESUMO
Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare, pigmented neoplastic entity of neural crest origin. Histological and immunohistochemical profiles indicate the presence of two components, small rounded neuroblast-like cellular areas and areas with large melanin-containing cells which consist of combination of neural, melanocytic, and epithelial cell types. Here we present two interesting cases of infants with MNTI which have different clinicopathological features. The first case is a 3-month-old female with rapidly growing MNTI involving the lacrimal sac and inferior wall of the orbital cavity, treated with total maxillectomy without orbital exenteration followed by chemotherapy. The second case is a 7-month-old male with slow-growing maxillary MNTI treated with complete surgical excision. In the female patient, histological findings revealed a predominance of neuroblast-like cellular areas and a high Ki67 index indicating rapid cellular proliferation. In the male patient however, large melanin-containing cells were dominant in this slow-growing tumor. These findings support the presence of two different types of MNTI, rapid-growing and slow-growing types, determined by the component of neuroblast-like cellular areas.
Assuntos
Neoplasias Oculares/diagnóstico por imagem , Doenças do Aparelho Lacrimal/diagnóstico por imagem , Neoplasias Maxilares/diagnóstico por imagem , Ducto Nasolacrimal/diagnóstico por imagem , Tumor Neuroectodérmico Melanótico/diagnóstico por imagem , Antineoplásicos/uso terapêutico , Neoplasias Oculares/metabolismo , Neoplasias Oculares/patologia , Neoplasias Oculares/cirurgia , Feminino , Humanos , Lactente , Antígeno Ki-67/metabolismo , Doenças do Aparelho Lacrimal/metabolismo , Doenças do Aparelho Lacrimal/patologia , Doenças do Aparelho Lacrimal/cirurgia , Imageamento por Ressonância Magnética , Masculino , Maxila/cirurgia , Neoplasias Maxilares/metabolismo , Neoplasias Maxilares/patologia , Neoplasias Maxilares/cirurgia , Ducto Nasolacrimal/metabolismo , Ducto Nasolacrimal/patologia , Ducto Nasolacrimal/cirurgia , Tumor Neuroectodérmico Melanótico/metabolismo , Tumor Neuroectodérmico Melanótico/patologia , Tumor Neuroectodérmico Melanótico/cirurgia , Exenteração Orbitária , Tomografia Computadorizada por Raios XRESUMO
Mesenchymal chondrosarcoma (MC) is an extremely rare subtype of chondrosarcoma that has a small round-cell sarcoma with focal cartilaginous differentiation, often with a pericytomatous vascular pattern. The non-cartilaginous components are usually dominant, and such lesions might be confused with other small round-cell tumors. Recently, a tumor-specific HEY1-NCOA2 fusion gene was identified in MC. Here we report the case of a 9-year-old boy who was diagnosed with MC by detection of HEY1-NCOA2 fusion signals in almost 50% of tumor cells in tissue sections on fluorescence in situ hybridization (FISH). In this way, the tumor was definitively diagnosed as MC. This case suggests that the detection of the HEY1-NCOA2 fusion gene on FISH is of diagnostic value for MC.
Assuntos
Fatores de Transcrição Hélice-Alça-Hélice Básicos/genética , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/genética , Proteínas de Ciclo Celular/genética , Condrossarcoma Mesenquimal/diagnóstico , Condrossarcoma Mesenquimal/genética , Fusão Gênica , Hibridização in Situ Fluorescente , Coativador 2 de Receptor Nuclear/genética , Tíbia , Criança , Humanos , MasculinoRESUMO
BACKGROUND: Epstein-Barr virus (EBV)-infected T or NK cells cause chronic active EBV infection (CAEBV). Allogeneic hematopoietic stem cell transplantation (HSCT) is curative treatment for CAEBV patients. However, chemotherapy prior to HSCT and optimal conditioning regimen for allogeneic HSCT are still controversial. PATIENTS AND METHODS: We retrospectively analyzed five patients with CAEBV treated with reduced-intensity conditioning (RIC) consisted of fludarabine, cyclophosphamide, and low-dose total-body irradiation followed by allogeneic bone marrow transplantation in a single institute. Only one of five patients received chemotherapy prior to transplantation. We analyzed EBV-infected cells in a patient whose EBV load increased after HSCT by T-cell repertoire assay, separation of T-cell subpopulations, in situ hybridization and microsatellite analysis. RESULTS: All five patients achieved engraftment, complete chimera, and eradication of EBV load. All patients have been alive without any serious regimen-related toxicity for more than 16 months following HSCT. However, one patient transplanted from HLA-matched sibling donor developed clonal proliferation of CD4+ Vß3+ T cells caused by monoclonal EBV infection on day 99 after transplantation. Further analysis revealed that the CD4+ Vß3+ T cells selectively harbored EBV genome, and these infected cells were derived from donor T cells. CONCLUSIONS: Allogeneic HSCT with RIC is a safe and effective treatment for better overall survival and less regimen-related toxicity in patients with CAEBV. Our first pediatric case reported in the literature suggests that we should consider the possibility of persistent EBV infection in donor T cells as well as the relapse in recipient cells if EBV load increases after allogeneic HSCT.
Assuntos
Infecções por Vírus Epstein-Barr/terapia , Transplante de Células-Tronco Hematopoéticas , Herpesvirus Humano 4 , Subpopulações de Linfócitos/virologia , Condicionamento Pré-Transplante , Adolescente , Antígenos CD4/metabolismo , Criança , Infecções por Vírus Epstein-Barr/imunologia , Infecções por Vírus Epstein-Barr/transmissão , Infecções por Vírus Epstein-Barr/virologia , Feminino , Herpesvirus Humano 4/genética , Herpesvirus Humano 4/imunologia , Humanos , Ativação Linfocitária , Subpopulações de Linfócitos/imunologia , Subpopulações de Linfócitos/metabolismo , Masculino , Receptores de Antígenos de Linfócitos T alfa-beta/metabolismo , Doadores de Tecidos , Transplante Homólogo , Resultado do Tratamento , Adulto JovemRESUMO
Autoimmune hemolytic anemia (AIHA) is a rare disease in infants, for which steroids are recognized as a first-line therapy for patients. Rituximab, a humanized monoclonal antibody raised against CD20, has been used in the treatment of autoimmune diseases, including AIHA, in adults and children. Due to limited follow-up study of the use of rituximab in the treatment for AIHA, its long-term efficacy, adverse effects, and immunological reconstitution of B cells have not been fully evaluated in infants. Here, we report a 3-month-old female patient with refractory AIHA, who was successfully treated with rituximab. Hemolytic anemia improved rapidly, and there were no severe adverse effects caused by rituximab. After 4.5 months following rituximab treatment, peripheral B cells were gradually reconstituted and required no intravenous immunoglobulin replacement thereafter. The patient has remained disease-free for more than 30 months without any additional treatment. This case suggests that rituximab may be a valuable therapeutic option, given its efficacy and minimal adverse effects in infants with therapy-resistant AIHA.
Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Anticorpos Monoclonais Murinos/administração & dosagem , Antineoplásicos/administração & dosagem , Anemia Hemolítica Autoimune/diagnóstico , Feminino , Humanos , Lactente , Indução de Remissão , Rituximab , Fatores de TempoRESUMO
Childhood cerebral ALD is a rapidly progressive and neurodegenerative disorder for which HSCT is the curative therapy if carried out at early stages. We successfully treated two patients of childhood cerebral ALD by CBT with RIC. The proband was a seven-yr-old boy whose brain MRI severity score (Loes score) was 14.5. Unrelated CBT was performed in five wk. To minimize conditioning regimen-related neurotoxicity, the combination of fludarabine (125 mg/m(2)), melphalan (140 mg/m(2)), and 4 Gy of brain-sparing TBI was used. The second patient was a six-yr-old brother of the proband. Four wk after the detection of a single small lesion (Loes score 1), he received unrelated CBT with the same RIC as the proband. In both patients, the engraftment was fast and stable, and severe complications were not observed. Furthermore, gadolinium-enhanced inflammation on brain MRI rapidly disappeared after CBT. Now, 20 and 13 months have passed after CBT, respectively, and both patients are neurologically stable. The RIC we used was sufficient for stable engraftment of cord blood and also tolerable even to the patient with advanced ALD. RIC-CBT should be considered for the patients with cerebral ALD at advanced stages, as well as those at early stages.
Assuntos
Adrenoleucodistrofia/cirurgia , Transplante de Células-Tronco de Sangue do Cordão Umbilical , Condicionamento Pré-Transplante/métodos , Adrenoleucodistrofia/diagnóstico , Encéfalo/patologia , Criança , Humanos , Masculino , Melfalan/administração & dosagem , Agonistas Mieloablativos/administração & dosagem , Índice de Gravidade de Doença , Vidarabina/administração & dosagem , Vidarabina/análogos & derivados , Irradiação Corporal TotalAssuntos
Antineoplásicos Fitogênicos/administração & dosagem , Vincristina/administração & dosagem , Coagulação Intravascular Disseminada/sangue , Coagulação Intravascular Disseminada/terapia , Hemangioma Capilar/sangue , Hemangioma Capilar/terapia , Humanos , Recém-Nascido , Síndrome de Kasabach-Merritt , Masculino , Dosagem RadioterapêuticaRESUMO
Although acquired idiopathic thrombotic thrombocytopenic purpura (ai-TTP) is rare in children, the authors present the case of a 9-month-old boy with ai-TTP showing severe deficiency of ADAMTS13 activity by its inhibitory IgG-autoantibody (4.8 Bethesda units/mL). Plasma exchange therapy was clinically effective but transient. Deficient activity of ADAMTS13 with the presence of its inhibitor persisted for 7 months after the initial diagnosis. However, other laboratory findings improved gradually with steroid (pulse) therapy. The hitherto insufficiently characterized clinical settings of ai-TTP during early childhood underscore the importance of measuring ADAMTS13 activity and its inhibitors for differential diagnosis in patients with thrombocytopenia of unknown etiology.
Assuntos
Proteínas ADAM/imunologia , Autoanticorpos/imunologia , Doenças Autoimunes/imunologia , Imunoglobulina G/imunologia , Púrpura Trombocitopênica Trombótica/imunologia , Proteínas ADAM/deficiência , Proteína ADAMTS13 , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/terapia , Terapia Combinada , Diagnóstico Diferencial , Humanos , Imunossupressores/uso terapêutico , Lactente , Masculino , Metilprednisolona/uso terapêutico , Transfusão de Plaquetas , Prednisolona/uso terapêutico , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/tratamento farmacológico , Púrpura Trombocitopênica Trombótica/terapiaRESUMO
Immune thrombocytopenic purpura (ITP) has been associated with several hematologic malignancies such as Hodgkin and non-Hodgkin lymphomas and chronic lymphocytic leukemia, but it is rare in children with acute lymphoblastic leukemia (ALL). Here, we report a 7-year-old girl with chronic ITP during early intensive phase of chemotherapy for ALL. She underwent splenectomy because thrombocytopenia had persisted even after treatment with intravenous immunoglobulin (IVIG), steroids, vincristine, rituximab, and anti-D antibody. After splenectomy, her platelet count had recovered, and maintenance therapy could be resumed with a support of IVIG. To our knowledge, this is the first child case of chronic ITP during chemotherapy for ALL and splenectomy was effective in this patient.