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The authors report a child younger than age 15 years with a rare hepatosplenic gamma/delta T-cell lymphoma, which is highly aggressive and primarily seen in young men. A 9-year-old girl presented with thrombocytopenia and hepatosplenomegaly. Bone marrow analysis revealed a metastatic pleomorphic lymphoma of peripheral T-cell phenotype, with rearrangement of the T-cell receptor gamma/delta and expression of CD3 and CD16/56. Instead of the previously reported primary, nonrandom, chromosomal abnormalities, isochromosome 7q and trisomy 8, this patient had four copies each of chromosome 7q, including isochromosome 7[i(7)(q10)] and der(21)t(7;21), as well as chromosome 8. This entity needs to be considered in women and children with lymphoma. Conventional therapy appears to be inadequate for cure.

This case suggests that typhlitis may cause delayed abdominal pathology. A history of this condition should be considered in the work-up of any patient with gastrointestinal or genitourinary pathology.

The authors report the concomitant occurrence of Duchenne muscular dystrophy (DMD) and alveolar rhabdomyosarcoma (RMS). A 4-year-old boy presented with symptoms involving his neuromuscular system that affected primarily his left hip and leg. Duchenne muscular dystrophy was diagnosed. Seven months later, metastatic alveolar RMS in the ipsilateral pelvis was documented. The diagnosis of one major disorder affecting striated muscle (DMD) may have prevented the early detection of another (RMS).

Purpose: A large cell anaplastic lymphoma that developed after treatment of a Ewing sarcoma (ES) is described., Patient: An 11-year-old girl with a pelvic ES developed a large cell, Ki-1+, anaplastic lymphoma in the same anatomic location 10 months after multimodal therapy., Results: ES recurred in the primary site 16 months after allogeneic marrow transplantation and 3.5 years after initial diagnosis, but the patient remains in remission from her lymphoma., Conclusion: The occurrence of lymphoma and ES in a short time interval in the same patient is very unusual. Whether etiologic factors other than chemoradiotherapy, including genetic disposition, play a role remains to be elucidated.

Purpose: Myelodysplastic syndromes in children commonly evolve into acute leukemia, usually acute myelogenous leukemia (AML) and rarely acute lymphoblastic leukemia (ALL). The lineage of the leukemia can be predicted based on characteristic morphologic and cytogenetic findings of the marrow and peripheral blood., Patient and Methods: A 3-year-old boy had refractory anemia with excess blasts and abnormalities suggestive of pre-AML with highly unusual cytogenetic changes. ALL of pre-B phenotype developed., Results: Leukoerythroblastic anemia, pseudo Pelger-Huet neutrophils, and dysmyelopoietic hyperplasia of the marrow suggested likely early progression to AML. Complex cytogenetic abnormalities (monosomy 17 and 20, ring chromosome 11 with deletion of bands q23, and a derivative dicentric chromosome 12) were present in both the myelodysplastic marrow and the subsequent ALL., Conclusion: This case presents cytogenetic evidence of common early progenitor cell ontogeny of both malignancies (refractory anemia with excess blasts and ALL).

Purpose: Agnogenic myeloid metaplasia (AMM) is a myeloproliferative disorder characterized by marrow fibrosis, extramedullary hematopoiesis, splenomegaly, and leukoerythroblastosis with abnormalities of red blood cell morphology. This is rarely encountered in children. No conventional curative therapy is known; however, allogeneic bone marrow transplantation (BMT) may eradicate the underlying stem cell abnormality with subsequent normal hematopoiesis., Results: We report a 3-year-old Arab boy who had AMM and who had normal marrow hematopoiesis and markedly reduced fibrosis after high-dose chemotherapy and matched sibling BMT., Conclusions: Allogeneic BMT offers a potential cure for patients with agnogenic myeloid metaplasia. A preparatory regimen containing busulfan and cyclophosphamide appears promising for patients with the disease.