Compound heterozygosity for Hb Lepore-Boston and Hb Neapolis (Dhonburi) [beta 126(H4)Val-->Gly] in a patient from Naples, Italy.

The simultaneous presence of two hemoglobin variants has been detected in a 14-month-old patient affected by thalassemia intermedia. The two variants were characterized by a combination of allele-specific amplification methods and mass spectrometric procedures carried out on isolated globins. These were identified as Hb Lepore-Boston and Hb Neapolis (also known as Hb Dhonburi) or beta 126 (H4)Val-->Gly. Hb Lepore-Boston is the most common hybrid variant detected in Campania and several cases of Hb Neapolis which causes a mild hypochromic microcytic anemia have been identified in this region in the last few years. This is the first report of a double heterozygosity involving Hb Lepore-Boston and Hb Neapolis.

Recombinant interferon alpha-2b as treatment of essential thrombocythaemia.

We report the disease characteristics and therapeutic results for 25 patients suffering from essential thrombocythaemia (ET), treated with recombinant interferon-alpha-2b (IFN-alpha 2b). ET was diagnosed according to the criteria of the Polycythaemia Vera Study Group. All patients were programmed to receive a subcutaneous induction treatment consisting of 3 MU of IFN-alpha 2b daily for 6 months. In responding patients, treatment was continued for a further 6 months with 3 MU of IFN-alpha 2b three times a week. Complete response was achieved in 13 of 25 patients, partial response in 10 of 25. In 2 cases, therapy was unsuccessful. Side effects were usually mild, consisting of flu-like symptoms in most cases, and were easily controlled by paracetamol. After a median follow-up of 14 months after discontinuation of the treatment, most patients retained the therapeutic response in the absence of toxicity.

Recombinant interferon alpha-2b in the treatment of polycythemia vera.

We studied the effects of recombinant alpha 2-b interferon (alpha 2-b IFN) in a dose of 3 x 10(6)U intramuscularly three times a week for 1 year in 13 patients affected by polycythemia vera (PV) previously treated with phlebotomy only. Response to treatment was evaluated by reduction of the number of phlebotomies required to retain normal hematocrit value. Ten out of 13 patients (77%) responded to treatment; in 4 of them the exigency of phlebotomy was completely eliminated. In all responders a concomitant decrease of platelet count and splenomegaly was obtained. Treatment was well tolerated and side effects were easily controlled. We conclude that alpha-IFN may represent an attractive therapeutic option in the management of the proliferative stages of PV.

Recombinant interferon-alpha 2A as maintenance treatment for patients with advanced stage chronic lymphocytic leukemia responding to chemotherapy.

Forty-five patients suffering from advanced B-CLL were randomized to receive interferon-alpha (IFN alpha) or no treatment after achieving complete remission or partial response, following a chemotherapy protocol called MiNa. The two groups were fully comparable in terms of clinical characteristics and level of response obtained by chemotherapy. IFN alpha was given at a dose of 3 megaunits three times a week intramuscularly for 1 year. The IFN-treated patient group showed a significantly longer duration of response and a less frequent incidence of infections as compared to the no treatment group. A minority of patients who had had partial response to chemotherapy obtained complete remission while on therapy with IFN alpha. Toxicity was mild and patient compliance was excellent. We conclude that IFN alpha may have a role as maintenance therapy in CLL for patients responding to chemotherapy.

Chronic lymphocytic leukemia coexisting with chronic myelomonocytic leukemia.

A case with coexisting chronic lymphocytic leukemia (CLL) and chronic myelomonocytic leukemia (CMML) is described. A 74-year-old man with a typical B-CLL also showed sustained peripheral blood and bone marrow monocytosis. Typical myelodysplastic changes and monosomy 7 were also found. Cytographic and immunophenotypic analysis confirmed the presence of two distinct cell populations, i.e., lymphoid and monocytoid. Both malignancies presented an extraordinarily benign prognosis. It remains uncertain whether monocytosis was either the expression of a distinct myelomonocytic clone or the progeny of a B/monocytic bipotential precursor able to feed both leukemic phenotypes.

Myeloid antigen expression in adult acute lymphoblastic leukemia: clinicohematological correlations and prognostic relevance.

Fifty adult patients with acute lymphoblastic leukemia (ALL) were prospectively studied to determine the clinical and hematological relevance of surface immunophenotypes. Before treatment, blast cells were assayed for reactivity to monoclonal antibodies to B-cell, T-cell, and myeloid (My) antigens. My antigens (CD13, CD33, and VIM2, singly or in combination) were demonstrated in 16 cases (32%) along with lymphoid specificities. Bone marrow and peripheral blood stains were classified according to French-American-British (FAB) Cooperative Group criteria and evaluated for myelodysplastic changes and azurophilic granules. Mean age of My+ patients was significantly higher. Furthermore, a greater number of My+ cases showed azurophilic cytoplasmic granules and acid ANAE positivity. FAB subtypes and myelodysplastic features did not significantly differ in the two groups analyzed, but patients with myelodysplastic abnormalities represented a significantly older age group. Response to treatment was comparable in My+ and My- cases, in terms of either complete remission rate or median survival duration.

Acute Myeloid Leukemia Expressing T-Cell Antigens: Clinico-Hematological Report on Six Cases.

In a series of 107 patients suffering from acute myeloid leukemia (AML), blast cells from six patients were found to simultaneously express CD2 and CD7 antigens along with CD13, CD33, and CDw 65 in various combinations. The frequency of the expression of both lymphoid markers recurred with a higher incidence than that anticipated by multiplying single antigens frequency. The clinical and hematologic features from CD2+/CD7 + AML patients were studied as well as compared with those of CD2-/CD7- AML patients observed in the same period. Morphologically, bone marrow smears from the AML hybrid subset showed a preponderant population of agranular blasts along with a minority of typical myeloid cells, characterized by larger amount of cytoplasm and, in three cases, by rare but distinct Auer Rods. In all cases more than 3% of blast cells were positive for myeloperoxidases and all samples were classified as M1 according to FAB classification. Clinically, CD2 + /CD7 + patients presented with a higher incidence of adenopathy and meningeal leukemia than did patients with CD2 + /CD7 - AML and were characterized by poor response to therapy in terms of both achievement and duration of remission. We conclude that simultaneous expression of CD2 and CD7 in AML is a non random event, recurring in more than 5% of cases and is associated with distinct clinical and hematologic features.

A new combination chemotherapy for advanced chronic lymphocytic leukemia (vincristine, cyclophosphamide, melphalan, peptichemio, and prednisone protocol).

The MiNa protocol (vincristine, cyclophosphamide, melphalan, peptichemio, and prednisone) was given to 20 patients with advanced B-cell chronic lymphocytic leukemia. Complete remission (absence of all clinical and bone marrow evidence of leukemia) was obtained in 35% of cases; partial response (greater than 50% reduction in organ enlargement and decreasement of lymphocyte count to less than 15(9) X 10/l) was observed in 35% of patients. Of 12 patients (60%) previously treated with chlorambucil and corticosteroids, nine responded to treatment. Toxicity was mild and the relapse rate particularly low. It was concluded that the MiNa protocol represents an effective chemotherapy for advanced and/or progressive CLL.

An analysis of morphological, cytochemical, immunological and clinical features of adult T cell acute lymphoblastic leukemia.

Morphological, cytochemical, immunological and clinical features of 19 adult patients with T cell acute lymphoblastic leukemia (T-ALL) were investigated at the time of diagnosis and were compared with those of 34 adult patients affected by B cell acute lymphoblastic leukemia (B-ALL). Immunophenotypic studies employing a wide panel of monoclonal antibodies (mAbs) revealed a heterogeneous pattern of antigen expression in the five T-ALL groups that were identified on the basis of blast T cell differentiation levels. PAS (periodic acid Schiff) negativity and focal AP (acid phosphatase) positivity, as well as white blood cell count and serum lactic dehydrogenase levels, were significantly related to T-ALL when compared with B-ALL. On the contrary, no statistically significant difference was demonstrated in the clinical outcome.

[Analysis of a case of multiple myeloma with late dissemination of skin nodules]. / Analisi di un caso di mieloma multiplo con disseminazione nodosa cutanea tardiva.

A case of a 55 years old woman suffering from multiple myeloma with strong bone marrow proplasmocytic infiltration, several osteolytic and osteoporotic lesions and high seric M-component level and hypertensive heart failure is described. After 32 months of partial remission obtained with cyclic chemotherapy, large cutaneous tumors arose. Despite of a new therapeutic trial, in the last 8 months, an increase of bone marrow and seric signs was observed without involvement of the lungs or kidneys or expression of plasma-cell leukemia. Death occurred at 50th month because of sepsis and heart failure. A real cutaneous tropism, late occurred and without cytohistological changes, is stressed. The meaning of the rich vascularization of the skin over the tumors in absence of inflammation and necrosis remains unclear.

[Prognostic evaluation of chronic lymphocytic leukemia in relation to the Rai classification. Critical analysis of 145 personal cases]. / Valutazione prognostica della linfoadenosi cronica in rapporto alla classificazione di Rai. Analisi critica di 145 casi personali.

145 patients suffering from Chronic Lymphocytic Leukemia, observed in the period from 1961 to 1976, were analyzed with regard to prognosis according to following factors: age, sex, Rai staging. Age and sex didn't show to influence median survival. According to Rai staging, and Authors, owing to obtained results, propose a classification in just two stages.