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.     

Non Hodgkin’s lymphoma seven years following remission of acute lymphoblastic leukemia

The authors describe a case of extramedullary relapse in lymph node presenting as lymphoblastic lymphoma seven years following remission of acute lymphoblastic leukemia. To the best of our knowledge, this is the first reported case of an isolated lymph node relapse with hematopoietic remission of leukemia. We have discussed cases of large cell lymphoma and other unusual areas of extramedullary relapse complicating acute lymphoblastic leukemia in hematopoietic remission.     

Acute Philadelphia chromosome-positive leukemia in an adolescent boy after liver transplantation

Acute leukemia is an untoward event following immunosuppression for solid organ transplantation and is related to the oncogenic effects of Epstein-Barr viral infections. The authors report a case of acute, Philadelphia chromosome-positive, T-cell lymphoblastic leukemia following liver transplantation. Molecular typing demonstrated a minor bcr-abl rearrangement (190 kD), which persisted in remission in 71% of peripheral neutrophils as determined by fluorescence in situ hybridization. The authors conclude that this patient may have presented in a lymphoid blast crisis of chronic myelogenous leukemia with an acute T-cell leukemia/lymphoma syndrome.     

Renal dysfunction and hyperuricemia at presentation and relapse of acute lymphoblastic leukemia

Hyperuricemia is an unusual presenting feature of acute lymphoblastic leukemia (ALL) and is generally associated with a large leukemic cell burden. We describe three children with T-cell ALL who presented with acute renal failure and very high serum uric acid concentrations, despite a relatively small leukemic cell burden. Two of the three patients had normal complete blood counts without circulating blasts or other physical evidence of leukemia. An isolated renal relapse in one case was associated with hyperuricemia, increased renal excretion of uric acid, and renal dysfunction. An unusually high rate of purine catabolism of the lymphoblasts may cause hyperuricemia in these cases. Unexplained hyperuricemia should prompt a search for occult malignancy.     

T-cell acute lymphoblastic leukemia following therapy of rhabdomyosarcoma.

Multiple studies have documented an increased risk of secondary malignancies in patients receiving alkylating agents. Secondary leukemia following chemotherapy accounts for about 20% of all secondary neoplasms; most are acute nonlymphocytic. Secondary acute lymphoblastic leukemia has rarely been reported in either adult or childhood cancer. We report the development of acute T-cell lymphoblastic leukemia in a child following successful treatment of a paravertebral embryonal rhabdomyosarcoma (ERS). Southern blot analysis of DNA extracted from the T-cell lymphoblasts, using probes homologous to loci on the short arm of chromosome 11; P-calcitonin, P40.1 and H-ras, did not demonstrate the chromosomal loss of heterozygosity (LOH), a common feature of embryonal rhabdomyosarcoma. The data presented support the assumption that de novo leukemia emerged following treatment of the primary malignancy.     

Acute lymphoblastic leukemia in a child with Wilson disease

Wilson disease is an autosomal recessively inherited disease of copper metabolism and is characterized by liver and central nervous system dysfunction. The heterozygote carrier state rate is about one in 90 persons and the incidence of the disease is about 30 in 1,000,000. Although leukemia is the most common form of childhood malignancies, the probability of the presence of Wilson disease and acute lymphoblastic leukemia in the same patient is very low. We report an unusual case of a child with Wilson disease who developed acute lymphoblastic leukemia in three months.     

ACUTE LYMPHOBLASTIC LEUKEMIA WITH COEXPRESSION OF CD56 AND CD57: Case Reports

The authors present the clinical profile of a 6-year-old girl with an unusual immunophenotype of acute lymphoblastic leukemia (ALL). At the initial presentation, massive hepatosplenomegaly developed. The leukemic cells were myeloperoxidase-negative and morphologically lymphoblastic. These cells were positive for B-precursor-cell (CD10, CD19) antigens and natural killer cells (CD56, CD57). Rearrangements of both immunoglobulin heavy chain alleles and monoallelic rearrangement of T-cell receptors (TCRs)-β and -δ genes, but not that of TCR-γ gene, were detected, suggesting that these cells being of B-precursor origin. The patient received chemotherapy for extremely high-risk ALL with a good response. To the authors' knowledge, this is the first pediatric case describing coexpression of CD56 and CD57 on B-lineage ALL.     

T-cell acute lymphoblastic leukemia following therapy of rhabdomyosarcoma

Multiple studies have documented an increased risk of secondary malignancies in patients receiving alkylating agents. Secondary leukemia following chemotherapy accounts for about 20% of all secondary neoplasms; most are acute nonlymphocytic. Secondary acute lymphoblastic leukemia has rarely been reported in either adult or childhood cancer. We report the development of acute T-cell lymphoblastic leukemia in a child following successful treatment of a paravertebral embryonal rhabdomyosarcoma (ERS). Southern blot analysis of DNA extracted from the T-cell lymphoblasts, using probes homologous to loci on the short arm of chromosome 11; P-calcitonin, P40.1 and H-ras, did not demonstrate the chromosomal loss of heterozygosity (LOH), a common feature of embryonal rhabdomyosarcoma. The data presented support the assumption that de novo leukemia emerged following treatment of the primary malignancy. © 1992 Wiley-Liss, Inc.     

Relapse of precursor B-cell acute lymphoblastic leukemia as an isolated central nervous system mass lesion 9 years after initial diagnosis

Seven years after completion of chemotherapy for acute lymphoblastic leukemia, diagnosed at the age of 5 years, a black female presented with signs of increased intracranial pressure. Neuroimaging showed a large enhancing extra-axial occipital tumor mass. The resection specimen showed morphologic, cytogenetic, and immunophenotypic features consistent with relapse of the primary leukemia. Bone marrow examination was negative for malignancy. The long duration of complete remission followed by the formation of a mass in the central nervous system are highly unusual features of recurrent acute lymphoblastic leukemia. © 1996 Wiley-Liss, Inc.     

Acute lymphoblastic leukemia with coexpression of CD56 and CD57: case report

The authors present the clinical profile of a 6-year-old girl with an unusual immunophenotype of acute lymphoblastic leukemia (ALL). At the initial presentation, massive hepatosplenomegaly developed. The leukemic cells were myeloperoxidase-negative and morphologically lymphoblastic. These cells were positive for B-precursor-cell (CD10, CD19) antigens and natural killer cells (CD56, CD57). Rearrangements of both immunoglobulin heavy chain alleles and monoallelic rearrangement of T-cell receptors (TCRs)-β and -δ genes, but not that of TCR-γ gene, were detected, suggesting that these cells being of B-precursor origin. The patient received chemotherapy for extremely high-risk ALL with a good response. To the authors' knowledge, this is the first pediatric case describing coexpression of CD56 and CD57 on B-lineage ALL.     

Unusual skeletal changes in acute lymphoblastic leukemia in children (author's transl)]

During the early phase of their disease three children with acute lymphoblastic leukemia showed unusual skeletal changes radiologically. Mainly osteolysis in the lower jaw, opacity of the sinus, decreased hight of the vertebrae, and a unilateral necrosis of the head of the femur. These skeletal alterations are much more common in other diseases than in acute lymphoblastic leukemia.     

Neutropenic acute acalculous cholecystitis (AAC) in a 12-year-old boy with T-acute lymphoblastic leukemia successfully managed with conservative treatment

Acute acalculous cholecystitis (AAC) is an inflammation of the gallbladder without the presence of gallstones. In children with malignancies or chemotherapy-induced neutropenia, AAC is very rare. Clinical diagnosis of AAC remains difficult in this patient population but an early recognition followed by an appropriate intervention may confer a benefit. Only three pediatric patients with underlying hematological malignancies whose clinical treatment course was complicated by the development of AAC have been described. We describe a neutropenic pediatric patient who developed AAC following chemotherapy for acute T-cell acute lymphoblastic leukemia (T-ALL), which was successfully managed with conservative treatment.

Abbreviations: AAC: Acute acalculous cholecystitis; T-ALL: T-cell acute lymphoblastic leukemia; TPN: Total parenteral nutrition     

Acute inclusion-body leukemia. Case report and review of literature

An unusual case of acute leukemia with basophilic cytoplasmic inclusion bodies in a child is described. Certain aspects of the case suggested a lymphoblastic phenotype and others a myeloid origin. The inclusions stained positively for acid phosphatase and toluidine blue. Too few patients have been reported to determine the prognostic significance of acute leukemia with inclusion bodies.     

A fatal case of acute pulmonary embolism caused by right ventricular masses of acute lymphoblastic lymphoma-leukemia in a 13 year old girl

We report a case of a 13-year-old girl with acute lymphoblastic lymphoma-leukemia, who presented with a cardiac metastasis in the right ventricle, resulting in a pulmonary embolism. At the time of her leukemia diagnosis, a cardiac mass was incidentally found. The differential diagnosis for this unusual cardiac mass included cardiac tumor, metastasis, vegetation, and thrombus. Empirical treatment was initiated, including anticoagulation and antibiotics. She underwent plasmapheresis and was administered oral prednisolone for her leukemia. Five days later, she experienced sudden hemodynamic collapse and required extracorporeal membrane oxygenation insertion and emergency surgery. These interventions proved futile, and the patient died. Pathology revealed that the cardiac mass comprised an aggregation of small, round, necrotic cells consistent with leukemia. This is the first known case of acute lymphoblastic leukemia presenting as a right ventricular mass, with consequent fatal acute pulmonary embolism. A cardiac mass in a child with acute leukemia merits investigation to rule out every possible etiology, including vegetation, thrombus, and even a mass of leukemic cells, which could result in the fatal complication of pulmonary embolism.     

Granulocytic Sarcoma Presenting as an Isolated Mediastinal Mass

ABSTRACT. An unusual case of granulocytic sarcoma presenting in an 8-year-old boy as an isolated mediastinal mass, preceding by 7 months the development of acute myeloid leukemia, is reported. The patient was initially treated for lymphoblastic lymphoma, with poor response. We recommend that in such cases, full histocytochemical and immunological characterization of the tumor should be performed.     

Granulocytic sarcoma presenting as an isolated mediastinal mass. A difficult diagnostic problem

An unusual case of granulocytic sarcoma presenting in an 8-year-old boy as an isolated mediastinal mass, preceding by 7 months the development of acute myeloid leukemia, is reported. The patient was initially treated for lymphoblastic lymphoma, with poor response. We recommend that in such cases, full histocytochemical and immunological characterization of the tumor should be performed.     

Establishment of leukaemia cell lines and their significance for clinical and experimental oncology (author's transl)]

Permanent in vitro growing leukemic cell lines have been established from all types of immunologically classified childhood leukemias. Essential characteristics of primary blasts and cultured cells are identical. In contrast to lymphoblastoid, non-leukemic cell lines, the Epstein-Barr-virus specific nuclear angiten (EBNA) is not detected. Up to now 8 Non-B-non-T cell lines (6 of them were derived from children with acute lymphoblastic leukemia, 2 from patients with chronic myeloid leukemia), 8 T-lines and one B-line have been established. Three Non-B-non-T lines from children with acute lymphoblastic leukemia (KM-3, RU-3, MH-3) and one T-cell line (JM) were cultivated by ourselves. Cultured blasts represent a pure tumor material which can be propagated in large quantities. Leukemic cell lines reveal a new approach for the search after leukemia-associated proteins and represent another possibility for the experimental investigation of the etiology of leukemia.     

Complete morphologic and molecular remission after introduction of dasatinib in the treatment of a pediatric patient with t-cell acute lymphoblastic leukemia and ABL1 amplification

T-cell acute lymphoblastic leukemia (ALL) accounts for 15% of ALL cases in children and has been associated with a worse prognosis. Cytogenetic studies show an abnormal karyotype in 50-60% of the T-cell ALL patients; ABL1 fusions are present in approximately 8% of the cases. Dasatinib, a second-generation tyrosine kinase inhibitor, directly targets the BCR-ABL gene. We describe a pediatric case of T-cell ALL with amplification of the ABL1 gene in which remission was achieved only after the addition of dasatinib to conventional chemotherapy.     

Mandibular relapse in acute leukemia. Report of a case

As chemotherapy has become more effective, patterns of relapse have begun to change. We report a patient with T-cell acute lymphoblastic leukemia (ALL) who had an isolated mandibular relapse that was first manifested as dental pain. Eight other recently reported patients with acute leukemia who relapsed in the mandible are reviewed. In four the relapse was isolated to the mandible; all four had ALL. Local control of disease was achieved with radiotherapy, often combined with chemotherapy. However, our patient and two of the other four subsequently relapsed and died of refractory leukemia.     

Acute encephalopathy and cerebral vasospasm after multiagent chemotherapy including PEG-asparaginase and intrathecal cytarabine for the treatment of acute lymphoblastic leukemia

A 7-year-old girl with an unusual reaction to induction chemotherapy for precursor B-cell acute lymphoblastic leukemia (ALL) is described. The patient developed acute encephalopathy evidenced by behavioral changes, aphasia, incontinence, visual hallucinations, and right-sided weakness with diffuse cerebral vasospasm on magnetic resonance angiography after the administration of intrathecal cytarabine. Vincristine, dexamethasone, and polyethylene glycol-asparaginase were also administered before the episode as part of induction therapy. Neurologic status returned to baseline within 10 days of the acute event, and magnetic resonance angiography findings returned to normal 4 months later.