Persistent polyclonal B lymphocytosis with multiple bcl-2/IgH rearrangements: a benign disorder.

The appearance of a chronic B lymphocytosis is usually associated with the existence of an underlying monoclonal malignant condition. However, a few cases of persistent polyclonal B cell lymphocytosis (PPBL), presented in young asymptomatic women with an uneventful course, have been reported in recent years. In these PPBL cases, since the lymphocytes usually display an anomalous morphology, a false diagnosis of a neoplastic chronic lymphoproliferative syndrome can be easily made. We report a typical case of PPBL that presents multiple bcl-2 rearrangements, the typical finding of follicular lymphomas. A review of different causes of benign non neoplastic lymphocytosis with special steadiness in changes in the lymphoid subsets will be made.

[Treatment with interferon alfa-2a in the chronic phase of Philadelphia-positive chronic myeloid leukemia]. / Tratamiento con interferón alfa-2a de la leucemia mieloide crónica Ph1 positiva en fase crónica.

PURPOSE: To evaluate the cytologic and cytogenetic response attained with interferon alpha-2a (IFN, Roferon*A) in patients with Ph '-positive chronic myelogenous leukaemia (CML) in the chronic phase. MATERIAL AND METHODS: A prospective study was carried out on 22 CML patients diagnosed in the Haematology Service at the Princesa Hospital in Madrid. The therapeutic regime consisted of two phases: A) Hydroxyurea was given until the white-cell count was reduced to 15-20 x 10(9)/L. B) Roferon*A was then given subcutaneously at a doses of 5 MU/m2 per day. The follow-up was performed weekly, and monthly once the leucocyte count had stabilized. The cytologic and cytogenetic response was assessed by bone marrow aspiration performed after 6, 9, 12 and 18 months. The toxicity was evaluated in accordance with the WHO recommendations. RESULTS: The median follow-up is 263 days (21-930). Thirteen patients (65%) had initial complete haematological response and 3 (15%) had partial response. The mean time to achieve response was 42 days (0-321). In the last evaluation, 69% of the patients were in sustained haematological remission (53% complete and 16% partial) with median follow-up of 232 days (21-930). The cytogenetic response was evaluable in 13 patients (follow up > or = 6 months): three attained complete response (23%) and three others partial response (23%). The commonest untoward effects were hypertriglyceridaemia (100%) and myelosuppression (86%). Grade-III thrombocytopenia was seen in 19% of the patients and grade-III anaemia or leucopenia in 5%. No infectious or haemorrhagic complications have appeared. Therapy was discontinued in 3 patients (14%), two due to severe flu-like syndrome and one for parkinsonism after 809 days of treatment. At the moment of evaluation two patients had died, one in lymphoid blastic crisis on day 217 and the other in the immediate post-BMT period. CONCLUSION: Treatment with interferon-alpha 2A is useful in the chronic phase of CML. An important number of responses can be attained, even in patients in the late chronic phase, and the toxicity seems acceptable.

Translocation (4;11) in ALL: a new morphologic aspect?

We have observed three patients with t(4;11)(q21;q23) among the 50 cases of ALL studied. Two were classified as L1 and the third as L3. We comment on the relationship between the morphologic classification of leukemias and the association with a t(4;11).

Empiric antibiotic therapy with an amikacin-carbenicillin-cefoxitin combination in granulocytopenic febrile patients--a clinical report.

The authors report 57 febrile episodes in 36 patients with leukemia and lymphoma. Patients with less than 1000 granulocytes and fever above 38.5 degrees C were included in this empirical antibacterial protocol (15 mg amikacin/kg/day/iv, 500 mg carbenicillin/kg/day/iv, 200 mg cefoxitin/kg/day/iv). The criteria for diagnosis of infection were those widely accepted [Schimpff et al. 1971]. Microbiologic documentation of infection was performed in 33.4% of febrile episodes. Antibacterial therapy induced an improvement in 75.1% of cases. Clinical response often occurred in the presence of profound granulocytopenia (in 72% of episodes). Therapy failure was higher in pneumonia (46%) and lower in fever of unknown origin (21%).

Double heterozygosis for hemoglobin C-beta thalassemia: description of a Spanish family. Hemoglobin C-beta thalassemia in a Spanish family.

A spanish family is described with two abnormal genes: 1) hemoglobin C in heterozygosis with normal hemoglobin, introduced by the subject's mother, and 2) heterozygotic betathalassemia for which the father is a carrier. In the subject and his sister, both abnormal genes coincide with the presence of hemoglobin C and hemoglobin F, simulating homozygosis for hemoglobin C. The clinical condition shows medium intensity chronic hemolysis. In the subject's brother, mother and grandmother, simple heterozygosis of hemoglobins A-C is seen, with no apparent clinical manifestations. The father shows subjaundice with some acute hemolytic episodes. Hypotheses are discussed which might explain the presence of these hemoglobins in Spain.