acid-phosphatase and Leukemia-Lymphoma--Adult-T-Cell

In a group of 21 children with T cell ALL we compared the clinical picture and laboratory finding in a subgroup of 13 patients with less mature (mCD3 negative) and a subgroup of 8 patients with mature (mCD3 positive) phenotypical type and the therapeutic response in relation to the stage of thymic differentiation of the blastic cells as well. We could not find any significant differences concerning the presence or absence of mediastinal thymic mass, organomegaly, WBC count, morphology of the blasts and their acid phosphatase and PAS reaction between cases with less mature and mature type of thymic differentiation. Concerning the therapeutic response, children with mature type of T-ALL have shown at 5 years significantly higher event free survival rate, in comparison with the group of patients with less mature type of T cells. Overall survival rate was also higher in the first group, but statistically not significant.

Topics: Acid Phosphatase; Adolescent; CD3 Complex; Cell Differentiation; Child; Child, Preschool; Combined Modality Therapy; Disease-Free Survival; Female; Humans; Immunophenotyping; Infant; Leukemia-Lymphoma, Adult T-Cell; Leukocyte Count; Male; Prognosis; Risk

Acid phosphatase (AP) reaction is a specific cytochemical marker for T-cell lymphoblastic leukaemia (T-ALL). Twenty six cases of ALL were diagnosed as T-ALL employing cytochemical profiles including AP and sheep red blood cell rosettes. Three district patterns of AP reaction were observed. On one end of the spectrum, 100% of the positive blasts showed the classical dot-like reaction while on the other 100% showed a scattered reaction. Between the two a mixed pattern was observed with 30-80% of the positive blasts showing the dot-like reaction while the rest showed scattered pattern. Dot-like pattern showed L1 morphology, high counts and low SRBC rosettes while scattered showed L2 morphology, low counts and high SRBC rosettes. The pattern observed in our series differs from the dot-like reaction observed in T-ALL in the western series. We recommend that this pattern must be borne in mind when employing AP reaction for T-ALL diagnosis (JPMA 42: 151, 1992).

Topics: Acid Phosphatase; Adult; Biomarkers, Tumor; Child; Humans; Leukemia-Lymphoma, Adult T-Cell; Leukocytes

Purine metabolism enzyme pattern, cytochemical markers and clinicopathologic features of common acute lymphoblastic leukemia antigen (cALLA; CD10)-positive, CD10-negative T acute lymphoblastic leukemia (ALL), and cALLA-positive non-T, non-B ALL (common ALL; C ALL) of children were compared. The results of immunophenotyping of blast cells in 61 children with ALL who were treated and followed during the last 7 years at the Second Pediatric Clinic in Bratislava are presented. The aim of our study was to determine the correlation of CD10 marker expression with purine enzyme activities and clinical course in ALL of children. Immunologic phenotype performed by a panel of monoclonal antibodies in indirect immunofluorescence assay revealed 3 main ALL groups: Common ALL (C ALL), T ALL and CD10+ T ALL (C + T ALL). An additional exact cytochemical marker analysis was performed in these three ALL immunologic subtypes. Two enzymes of purine metabolism, i.e. adenosine deaminase (ADA) and purine nucleosidephosphorylase (PNP) were investigated in blast cells by paper radiochromatography. Life-table analysis revealed significant prognostic differences with regard to event-free survival and overall survival in followed groups of ALL patients. Our results showed a rather high frequency of mixed (C + T) ALL phenotype. The characteristic T ALL enzyme pattern (high ADA, low PNP) was present not only in T, but also in CD10+ T ALL blast cells. The T cell marker showed to be dominant in the determination of clinical course and prognostic significance in children with ALL; children with T and CD10+ T ALL phenotype, in contrast to C ALL phenotype, experienced more frequent relapses and a shorter event-free survival.

Topics: Acid Phosphatase; Adenosine Deaminase; Adolescent; Antigens, Differentiation; Antigens, Neoplasm; Child; Child, Preschool; Female; Humans; Infant; Leukemia-Lymphoma, Adult T-Cell; Male; Neprilysin; Phenotype; Purine-Nucleoside Phosphorylase; Survival Rate

We describe two childhood cases of a T-cell malignancy indistinguishable from nonendemic, idiopathic adult T-cell leukemia/lymphoma. The cases illustrate the heterogeneity of nonendemic, idiopathic ATLL, including the presence of mediastinal masses and more heterogeneous immunologic markers than endemic cases, and represent the two youngest cases of ATLL yet described. The differential diagnosis of nonendemic, idiopathic ATLL is discussed.

Topics: Acid Phosphatase; Adolescent; Biomarkers; Bone Marrow; Child; Diagnosis, Differential; Female; Humans; Karyotyping; Leukemia-Lymphoma, Adult T-Cell; Lymph Nodes; Male; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Skin