Trilineage response to granulocyte colony-stimulating factor administration in a patient with myelodysplastic syndrome.

We report a 72-year-old man with refractory anemia with excess of blasts who presented severe pancytopenia and pneumonia and received granulocyte colony-stimulating factor (G-CSF) treatment over a 6-week period. In addition to a dramatic increase in mature neutrophils, platelet count and hemoglobin level, the patient achieved a hematological remission which continued for more than 5 months despite discontinuation of the treatment. This observation confirms that in some cases during G-CSF treatment erythropoiesis and thrombopoiesis may improve in addition to the expected effect on neutrophils. While the patient remained in hematological remission, bone marrow examination revealed trilineage dysplasia. This finding suggests that the hematological remission in this patient may not have resulted from a recovery of non-clonal hematopoiesis of a normal clone, but may have derived instead from the monoclonal hematopoiesis of a neoplastic clone.

[Churg-Strauss vasculitis in a 53-year-old man]. / Vasculite di Churg-Strauss in un uomo di 53 anni.

The Authors describe a 53 year old patient, who, after suffering from asthma over the past 10 years, presented with abdominal pain, diarrhoea, dyspnea, petechias on lower limbs, and subsequently developed fever, polyneuritis, pericardial effusion and renal failure. Laboratory showed elevated IgE, presence of antinuclear antibodies to DNA, serum rheumatoid factor and peripheral eosinophilia. The clinical course was suggestive for systemic vasculitis; lung, skin biopsies and renal angiography confirmed this diagnosis: the association with asthma and eosinophilia fulfill the diagnosis of allergic angiitis and granulomatosis (Churg-Strauss syndrome).

Reactive hemophagocytosis in Ki-l positive large cell lymphoma: a case study.

A case of large cell lymphoma presenting with hemophagocytic syndrome is reported. The clinicopathological findings suggested a diagnosis of malignant histiocytosis, but on the basis of immunohistological studies Ki-l lymphoma was diagnosed. Neoplastic cells expressed activation antigens such as HLA-DR, IL 2R, T10 and Ki-l, and showed high proliferative activity, but were devoid of T and B cell markers. The high percentage of reactive macrophages found in the bone marrow and lymph node probably reflected the release of lymphokines by the tumor population. The patient was treated with aggressive chemotherapy and is in complete remission at 8 months from diagnosis.

Cytochemistry of dipeptidylaminopeptidase IV in T lymphoblastic leukaemia-lymphoma.

The cytochemical distribution of dipeptidylaminopeptidase IV (DAP IV) was studied in 5 cases of T lymphoblastic leukaemic lymphoma and 12 cases of acute T lymphoblastic leukaemia, in order to ascertain differences between the enzyme positivity patterns of T cells at different stages of differentiation. Early thymic phenotype cases were almost completely negative; those of intermediate and mature thymic phenotype showed positivity in various percentages of blasts: either a single coarse granule or many coarse and small granules were detected. In mature phenotype cells a particularly intense DAP IV reaction was observed. In conclusion, our findings suggest that DAP IV reaction could be a useful tool for the cytochemical characterization of T acute leukaemia subtypes.

Cytochemical profile of lymphocytes in haemophilia patients at risk of AIDS.

We report the cytochemical and immunologic features of lymphocytes of 31 patients (26 with haemophilia A and 5 with haemophilia B) chronically treated with commercial clotting factor concentrates. 6 patients, asymptomatic for AIDS were anti-HIV positive. In comparison to aged cross-matched male controls, significant increases of absolute number of lymphocytes and of T and B cell subpopulations with significant reduction of the percentages of CD3, CD4, CD2, CD5, sIg, and CD16 cells was found in haemophiliacs. PAS reaction showed a lower grading score in haemophiliacs and the staining for NBE, AP, NABG revealed a significant reduction of the percentage of the lymphocytes with dot-like positivity in the same patients. The percentage number of AP reactive lymphocytes with dot-like positivity was directly related to the percentage of CD3, and CD4 cells. The anti-HIV positive patients presented lower percentages of CD4 and of dot-like reactive lymphocytes.

Tetrahydrofolate dehydrogenase cytochemistry in acute lymphoblastic leukemia.

We studied the cytochemical distribution of tetrahydrofolate dehydrogenase (FH4D), an enzyme involved in nucleic acid metabolism and thus in cell proliferation and differentiation processes, in bone marrow blasts from 37 cases of acute lymphoblastic leukemia (ALL), of whom 23 were pediatric patients. 26 cases were analyzed at onset, 11 in relapse. The ALL cases were immunologically classified as T (10), common (20), B (3) and null (4). In each subgroup the majority of lymphoblasts were positive, with heterogeneous positivity patterns and variable degrees of enzyme activity. Most T lymphoblasts were characterized by focal localization of FH4D, whereas in common blasts reactivity - usually less strong - was either focally localized or scattered with several fine granules. Finally, many B and null blasts showed diffuse positivity. A quantitative evaluation of FH4D activity using cytophotometric technique (Vickers M86) demonstrated higher degrees of reactivity in leukemic blasts than in normal lymphocytes. Moreover, slightly different levels of reactivity were observed in relation to immunological phenotype, age and stage of the disease. Therefore we think that FH4D is a useful additional marker for ALL characterization.

Cytochemistry of N-acetyl-beta-glucosaminidase in normal and leukemic T cells.

A cytochemical study of the N-acetyl-beta-glucosaminidase (NABG) activity in normal and leukemic T cells was carried out to ascertain any relationships between cytochemical reactivity and membrane phenotype. Under normal conditions, most T4-positive cells, defined on the basis of monoclonal antibodies and immunogold, were characterized by focal reactivity. In chronic T lymphoproliferative disorders, the reaction product of T4-positive cells consisted of a single coarse granule, while T8-positive cells demonstrated many scattered granules. In all cases of acute T cell malignancies, lymphoblasts were positive with a single coarse granule or many coarse and small granules located at one pole of the cell.