Lymphoblastic transformation of chronic myelomonocytic leukemia in an infant

Yamamoto, Masuji; Nakagawa, Mitsuo; Ichimura, Noriko; Ohtsuki, Fumiko; Ohtsuka, Yoshitoshi; Tsujino, Yoshiaki; Tanaka, Aiichiro; Kamiya, Takashi; Wada, Hiroyoshi

doi:10.1002/(sici)1096-8652(199607)52:3<212::aid-ajh14>3.0.co;2-h

Cited by 13 publications

(5 citation statements)

References 9 publications

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“…9 There are 2 other reports of transformation of childhood MDS into ALL, one from refractory anemia with excess blasts and the other from chronic myelomonocytic leukemia. 10,11 Our patient had been on cyclosporine therapy for a period of 9.5 months before the development of acute leukemia. Cyclosporine was introduced in treatment of MDS first in 1998.…”

Section: Discussionmentioning

confidence: 99%

Transformation of Childhood MDS-refractory Anemia to Acute Lymphoblastic Leukemia

Goel

Kumar²,

Bakhshi³

2007

Journal of Pediatric Hematology/Oncology

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Myelodysplastic syndromes (MDS) are clonal disorders of hematopoietic stem cell. Patients have a deteriorating course with about 30% evolving into acute leukemias usually of the myeloid phenotype. Evolution into acute lymphoblastic leukemia is a rare and intriguing phenomenon seen in far less than 1% of adult cases, and extremely rare in pediatric population. We report a case of childhood MDS-refractory anemia transforming into acute lymphoblastic leukemia after an interval of 21 months since presentation and being on cyclosporine therapy for 9.5 months. The case raises further questions about the biology of MDS and the potential role of cyclosporine in leukemic transformation.

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Section: Discussionmentioning

confidence: 99%

Transformation of Childhood MDS-refractory Anemia to Acute Lymphoblastic Leukemia

Goel

Kumar²,

Bakhshi³

2007

Journal of Pediatric Hematology/Oncology

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“…In addition, two cases of JMML evolving into B lymphoid blast crisis have been reported. 35,36 A study of immunoglobulin heavy chain gene rearrangements also supported clonal derivation of B lymphoblasts in some patients. 37 Taken together, these data suggest that in JMML the clonal involvement of cells of the lymphoid lineage, in particular B lymphocytes, is heterogeneous.…”

Section: Discussionmentioning

confidence: 94%

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1999

Leukemia

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Juvenile myelomonocytic leukemia (JMML) is a malignant hematopoietic disorder of early childhood with excessive proliferation of the myeloid and monocytic lineage. Deregulation of the RAS signal transduction pathway is thought to play a key role in its pathogenesis. We examined peripheral blood or bone marrow cells of 36 children with JMML for activating point mutations in codons 12, 13 and 61 of the NRAS and KRAS proto-oncogenes by allele-specific restriction assay, single-strand conformation polymorphism and/or direct sequencing. Codons 12, 13 and 61 of HRAS were examined in 26 of these patients. We detected RAS mutations in six cases (17%) located at N12 (n = 2), N13 (n = 3) and K13 (n = 1). In addition, we performed clonality studies on different cell lineages in four of these patients applying the RAS mutation, the karyotype and X-chromosome inactivation patterns as clonal markers. Erythroid cells carried mutant RAS, indicating clonal origin. In EBV B cell lines, one of three patients studied harbored a RAS mutation, while the other two patients had polyclonal B cells with wild-type RAS. T lymphocytes were examined in one patient; they were polyclonal and had wild-type RAS. It is likely that JMML is a heterogeneous disease with respect to clonal involvement of different lineages.

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“…Nonetheless remarkable is the further evolution of the disease with development of T-lineage ALL with typical T-cell leukemia features, including involvement of the mediastinum, central nervous system (CNS), and testes. There are only 4 descriptions of lymphoblastic transformation of JMML available through the Medline database, [10][11][12][13][14] and few additional patients have been mentioned in reviews. Among these cases, only 1 patient had a T-cell lymphoma.…”

Section: Discussionmentioning

confidence: 99%

“…9 Other than progression to a myeloid blast crisis, a few cases of transformation in B-lineage lymphoblastic leukemia and 1 case of T-lineage non-Hodgkin lymphoma have been described in patients with JMML, further suggesting the stem cell origin of the disease. [10][11][12][13][14] Herein we describe the first case of overt T-lymphoblastic leukemia arising in a patient after CR of JMML, which was achieved after treatment with 13-cis RA+AraC and lasted 7 years with no maintenance therapy.…”

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confidence: 99%

Development of T-cell Acute Lymphoblastic Leukemia in a Patient in Very Long Lasting Complete Remission of Juvenile Myelomonocytic Leukemia

Maschan¹,

Хачатрян²,

Solopova³

et al. 2011

Journal of Pediatric Hematology/Oncology

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Juvenile myelomonocytic leukemia (JMML) occurs with an incidence of 1.2 per million children a year, and represents 18% to 30% of all myelodysplastic (MDS) and myeloproliferative (MPS) disorders in the age group below 15, being by far the most common MDS/MPS in children younger than 4 years. The only therapeutic approach which results in a definitive cure of patients with JMML is myeloablative chemo-therapy/radio-therapy, followed by allogeneic hematopoietic cell transplantation. Few cases of transformation of JMML in acute lymphoblastic leukemia have been reported. We describe a child with JMML diagnosed at the age of 4 months in whom complete remission was achieved with 13-cis retinoic acid and cytosine-arabinoside and was sustained for 7 years with no maintenance therapy. Ninety-eight months after the diagnosis of JMML was established, overt T-cell leukemia developed. Treatment with acute lymphoblastic leukemia (ALL)-directed chemotherapy induced complete restoration of normal hemopoiesis, but testicular involvement persisted. The patient died after transplantation with unrelated cord blood. This case suggests that JMML is a true stem cell disorder and that stem cell transplantation should be considered, even in patients with a very favorable clinical course.

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Lymphoblastic transformation of chronic myelomonocytic leukemia in an infant

Cited by 13 publications

References 9 publications

Transformation of Childhood MDS-refractory Anemia to Acute Lymphoblastic Leukemia

Transformation of Childhood MDS-refractory Anemia to Acute Lymphoblastic Leukemia

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Development of T-cell Acute Lymphoblastic Leukemia in a Patient in Very Long Lasting Complete Remission of Juvenile Myelomonocytic Leukemia

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