T-cell acute lymphoblastic leukemia relapsing as acute myelogenous leukemia.

We present the unusual case of a 16-year-old girl with T-cell acute lymphoblastic leukemia (ALL) with an early thymocyte immunophenotype without myeloid markers, who after 13 months of complete hematological remission relapsed as acute myelogenous leukemia (AML) with minimal differentiation and died of her disease. Whether the AML represented a relapse with lineage switch of the original immature T-cell clone or a new secondary malignancy, could not be proven due to the absence of molecular or clonal markers. This report suggests that a subset of CD7+ T-cell leukemias without mature T-cell antigens (CD4-, CD8-) are minimally differentiated and can relapse as AML.

Neurofibromatosis type I and smoldering multiple myeloma: a case report.

We present a case of a 64-year-old woman with neurofibromatosis (NF1) and smoldering multiple myeloma (SMM). SMM was diagnosed 9 years ago when the asymptomatic patient was found to have mild anemia, IgA paraproteinemia, hypogammaglobulinemia, osteopenia without any lytic bone lesions and bone marrow plasmacytosis. During follow-up period she remained stable in an excellent clinical condition without requiring any therapy for almost 4 years. Forty-two months after diagnosis she had a femoral fracture and since then biphosphonates have been administered intravenously, once monthly. Subsequent evaluations of the disease showed a dramatic reduction of IgA paraprotein to below half the initial value. We will discuss the probable pathogenesis of plasma cell dyscrasia in NF1 patients, as well as the likely antimyeloma activity of biphoshonates.

Fulminant hepatitis due to varicella zoster virus in a girl with acute lymphoblastic leukemia in remission: report of a case and review.

The authors describe a 4-year-old girl with acute lymphoblastic leukemia in remission who developed fulminant hepatic failure due to varicella-zoster virus (VZV). Diagnosing VZV visceral infection in immunocompromised patients is often difficult due to atypical clinical presentation with few or no skin lesions and severe abdominal or back pain. Prompt initiation of empirical treatment with acyclovir and VZV immunoglobulin pending results of the serum polymerase chain reaction for VZV is warranted in this clinical setting.

Rituximab for refractory Evans syndrome and other immune-mediated hematologic diseases.

The authors describe a 21-year-old man with long-lasting Evans syndrome refractory to corticosteroids and immunosuppressive agents; the patient responded to four weekly infusions of rituximab. The patient relapsed with thrombocytopenia 7 months post-therapy and was successfully re-treated with two weekly doses of the same monoclonal antibody. He remains in remission for 7-plus months after the second treatment. Therapy was well tolerated, and no infectious complications occurred, despite avoiding administration of prophylactic gammaglobulin. Rituximab appears safe and modestly effective in a variety of immune-mediated hematologic diseases, including autoimmune hemolytic anemia, chronic immune thrombocytopenia, Evans syndrome, pure red cell aplasia, mixed type II cryoglobulinemia, cold agglutinin disease, and Waldenstrom's macroglobulinemia. However, as most of the published literature consists of case reports and small case series, international collaboration is essential in order to better define the efficacy and safety of this agent in children and adults with hematologic diseases.

Amifostine as differentiating agent in cord blood and bone marrow cultures from children with hematological disorders.

Amifostine protects normal tissues from the cytotoxicity of chemotherapy and radiotherapy and has an effect a growth factor both in vivo and in vitro. To evaluate its possible hemopoietic promoting activity, the authors studied the effect of WR-2721 on cord blood and bone marrow progenitors from children with hematological disorders and normal controls in semisolid cultures after preincubation with 0.1-1000 microM amifostine. Amifostine enhanced the growth of BFU-E in autoimmune neutropenia but inhibited CFU-GM development. It also exerted an inhibitory effect upon growth of committed progenitors in the groups studied (acute lymphoblastic leukemia, Langerhans cell histiocytosis), including cord blood and marrow of healthy controls.

Remission of a chiasmatic glioma in a non-NF1 patient after brief chemotherapy with vincristine and carboplatin: case report and literature review.

We describe the case of an 8-year-old girl without neurofibromatosis, who presented with total loss of vision on the left eye, due to a chiasmatic mass with imaging characteristics of glioma, accompanied by a second asymptomatic mass in the middle cranial fossa, along the intracranial route of the right trigeminal nerve. The patient received a total of 10 weekly injections of vincristine and four injections of carboplatin every 3 weeks and achieved a very good partial response (97% volume reduction) after the nineth week of therapy with acceptable toxicity. Given the natural history of opticochiasmatic gliomas, we cannot rule out the possibility of a spontaneous regression. However, we believe the quick response accompanied by visual improvement was most likely due to chemotherapy. A trial of vincristine and carboplatin may be worthwhile in children with symptomatic chiasmatic gliomas, irrespective of their age.

Infectious toxicity of dexamethasone during all remission-induction chemotherapy: report of two cases and literature review.

Traditionally, children with acute lymphoblastic leukemia receive prednisone, as part of multiagent remission-induction chemotherapy. Recently, many cooperative groups use dexamethasone instead of prednisone during induction. We describe the infectious toxicities experienced by the first two patients in our institution treated with dexamethasone (10 mg/m(2)/day for 4 weeks with gradual tapering) during induction according to the dexamethasone arm of BFM 2000 and review the relevant literature that suggests an increased risk of infectious complications with dexamethasone. Only a prospective two-arm ALL dexamethasone study at two dose levels (6 and 10 mg/m(2)/day) will clarify if indeed the higher dose of dexamethasone during induction is more effective and without unacceptable toxicity.

Capnocytophaga gingivalis bacteremia detected only on quantitative blood cultures in a child with leukemia.

Capnocytophaga species are inhabitants of the normal mouth flora. We describe the case of a 6-year-old-girl with leukemia and poor oral hygiene who developed bacteremia caused by Capnocytophaga gingivalis. The organism was detected only on quantitative blood cultures.

Three cases of viridans group streptococcal bacteremia in children with febrile neutropenia and literature review.

Three cases of viridans group streptococcal bacteremia in 2 children with brain tumours and in 1 with autoimmune neutropenia are presented. All children were neutropenic. The 2 patients with malignancy also had mucositis. The isolated strains of viridans group streptococci showed considerable resistance to antibiotics. All patients were empirically treated with third generation cephalosporins and amikacin, before antibiotic sensitivities were available and recovered without complications. Viridans group streptococcal bacteremia should be suspected in neutropenic children, especially in the presence of mucositis. Prospective, randomized clinical trials of mouth antiseptics are needed to clarify the usefulness, if any, of such measures in the reduction of viridans group streptococcal bacteremia in this group of patients.