ABSTRACT
The authors describe 10 cases of myelofibrosis diagnosed and managed at their center over 16 years. There were 2 and 8 cases, respectively, of primary and secondary myelofibrosis. All patients presented with fever, pallor, hepatosplenomegaly, and/or lymphadenopathy. Hodgkin's lymphoma (n = 4), neuroblastoma (n = 1), thrombasthenic thrombopathy (n = 1), and retroperitoneal-mass (n = 1) were causal in 7 patients, whereas the diagnosis could not be established in a sole case of secondary myelofibrosis. Patients were managed with chemotherapy and appropriate care. However, outcome was poor. The authors emphasize variable clinical-laboratory spectrum of myelofibrosis, highlight management concerns, and demonstrate that prognosis/outcome depends upon appropriate management of the underlying condition.
Subject(s)
Primary Myelofibrosis/drug therapy , Primary Myelofibrosis/etiology , Child , Disease Management , Drug Therapy , Hodgkin Disease/complications , Humans , India , Neuroblastoma/complications , Primary Myelofibrosis/diagnosis , Retroperitoneal Neoplasms/complications , Thrombasthenia/complications , Treatment OutcomeABSTRACT
This retrospective analysis of 254 children less than 15 years of age treated with MCP-841 protocol from June 1992 to June 2002 was undertaken to identify the pattern of relapse and determine management lacunae. Two hundred twenty-three (87.8%) children achieved a complete remission of whom 40 (17.9%) relapsed. The mean age of relapsed patients was 6.5 years. The male/female ratio was 9:1. There were 23 (57.5%) isolated bone marrow (BM), 7 (17.5%) isolated central nervous system (CNS), 2 (5%) isolated testicular, 5 (12.5%) BM+testes and 1 each of BM+CNS, CNS+testes, and isolated bone relapses. Twenty-seven children (67.5%) relapsed on-therapy whereas 13 (32.5%) relapsed posttherapy. All 9 CNS relapses occurred on-therapy whereas 5/8 (62.5%) of testicular relapses occurred posttherapy. Lymphadenopathy was the only significant predictor for relapse. High-risk features such as age less than 1 year and greater than 10 years (P=0.047) and white cell count greater than 50.0 x 10(9)/L (P=0.044) were significantly more frequent in patients with early on-therapy relapse than in patients with off-therapy relapse. The overall survival in the entire study cohort was 67+/-3.5%. Modest survival outcome, relapse while on chemotherapy and the higher incidence of CNS and testicular relapse indicate the need for reappraisal of our treatment protocol. There is a need of identifying risk factors and high-risk groups in our set of patients and risk-stratified intensification of chemotherapy in them.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Marrow Neoplasms/therapy , Brain Neoplasms/mortality , Neoplasm Recurrence, Local/therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Testicular Neoplasms/therapy , Adolescent , Bone Marrow Neoplasms/mortality , Brain Neoplasms/therapy , Child , Child, Preschool , Cohort Studies , Combined Modality Therapy , Female , Humans , Infant , Male , Neoplasm Recurrence, Local/mortality , Precursor Cell Lymphoblastic Leukemia-Lymphoma/mortality , Radiotherapy Dosage , Remission Induction , Retrospective Studies , Survival Rate , Testicular Neoplasms/mortality , Treatment OutcomeABSTRACT
In this letter the authors describe an acute lymphoblastic leukemia survivor with pontomedulary hemorrhage due to basilar artery malformation. Pathogenesis and management are discussed.
Subject(s)
Antimetabolites, Antineoplastic/adverse effects , Intracranial Arterial Diseases/chemically induced , Intracranial Hemorrhages/chemically induced , Methotrexate/adverse effects , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Antimetabolites, Antineoplastic/administration & dosage , Arteries/abnormalities , Child, Preschool , Combined Modality Therapy , Humans , Injections, Spinal , Intracranial Arterial Diseases/diagnostic imaging , Intracranial Hemorrhages/diagnostic imaging , Male , Methotrexate/administration & dosage , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnostic imaging , Radiography , Radiotherapy DosageABSTRACT
Pyoderma gangrenosum is a neutrophilic dermatosis that may occur idiopathically or in association with various systemic diseases and malignancy. Although the association of this entity with myeloid malignancies is well known, its association with lymphoid malignancy is extremely rare. We describe atypical pyoderma gangrenosum in association with acute lymphoblastic leukemia in a 2-year-old child, an occurrence not reported before.
