Jl. Michaux

Rearrangements of the Short Arm of Chromosome No-6 in T-cell Lymphomas

Cytogenetic studies on four patients with T-cell lymphomas are reported. In all four the short arm of chromosome No. 6 (6p) was abnormal. In three cases it was involved in a translocation, and in one there was a deletion of the terminal part: del(6)(p23-24). One of the translocations could be identified as a t(2:6)(q24;p24). Identification was not possible in the others. These 6p anomalies were associated with complex structural and numerical abnormalities of other chromosomes in three out of four cases. In three patients chemo- and/or radiotherapy administration preceded cytogenetic investigations.

Trisomy 3 is a consistent chromosome change in malignant lymphoproliferative disorders preceded by cold agglutinin disease

Summary. Cold‐antibody autoimmune haemolytic anaemia is a rare entity that has been associated with a wide variety of pathological processes, including malignant lymphoproliferative disorders. In this retrospective study we recorded, as far as possible, clinical, haematological, immunological, morphological, pathological, cytogenetic and molecular data on 10 patients with cold agglutinin disease (CAD). Cytogenetic anomalies were found in four cases in which an underlying lymphoma could be evidenced. Trisomy 3 was the only recurrent aberration in our series. It was observed in all patients with abnormal karyotype, either as a complete trisomy or as a partial trisomy of the long arm. The importance of this particular karyotypic aberration in the monitoring of CAD is emphasized.

Specificity and sensitivity of immunocytochemistry for detecting P-glycoprotein in haematological malignancies.

AIMS--To determine the optimal working conditions of the alkaline phosphatase-antialkaline phosphatase (APAAP) method to establish a specific and sensitive assay for the detection of low numbers of MDR positive cells in patients with hematological malignancies. METHODS--Three monoclonal antibodies (C-219, JSB-1, MRK-16) were used for the detection of P-glycoprotein (P-gp) in cell lines and in samples from 43 patients with haematological malignancies. The results of the APAAP method were compared with western blotting for specificity and sensitivity. RESULTS--Excellent correlation was obtained between optimised APAAP and western blotting, except in the case of multiple myeloma. JSB-1 seemed to be the more useful monoclonal antibody for the APAAP which was more sensitive than western blotting in its ability to detect single P-gp positive cells. CONCLUSIONS--Methods for P-gp detection, as defined by multidrug resistant (MDR) cell lines, are not necessarily optimal and specific for clinical samples and may lead to higher false positive and negative results, according to the conditions and the monoclonal antibodies used.

Apparent focal bone marrow ischemia in patients with marrow disorders: MR studies.

Objective We sought to assess with MR the focal bone marrow abnormalities in patients with chronic marrow disorders and acute limb pain. Materials and Methods We investigated and followed with MRI four patients with proliferative or dysplastic marrow disorders presenting with acute but spontaneously resolving hip or thigh pain. Results Ten focal marrow lesions were demonstrated on T2-weighted images as high signal intensity (SI) areas. They remained undetected on T1-weighted images as they showed a low SI similar to the disease-related low SI of the entire marrow. Postcontrast images demonstrated lack of enhancement in the lesions. Follow-up enhanced MR images showed intense enhancement within the lesions, while unenhanced MR images remained unchanged. Later on, these focal marrow abnormalities completely resolved. These lesions most likely represent bone marrow ischemia, although histological proof is lacking. Conclusion Acute bone pain in patients with bone marrow disorders may be related to focal marrow lesions suggestive of bone marrow ischemia.

Quantitative Assessment of Erythropoiesis in Bone-marrow Expansion Areas Using Fe-52

Summary. Quantitative 52Fe scans were performed in 180 patients. Expansion of bone marrow was observed in 70. This bone marrow expansion was a nearly constant feature in haemolytic anaemia and in sideroblastic anaemia. It occurred in a third of the patients with myelofibrosis. In patients with polycythaemia rubra vera, expansion was noticed in only two out of seven. Erythropoiesis in expansion areas occurred despite persistence of fat in the iliac crest bone marrow biopsy. It could exist with a slight increase in erythropoiesis and might develop only after a long period of erythropoietic stimulation.Increased marrow activity can take place without erythropoietic expansion in long bones. The fraction of iron uptake in expansion areas did not exceed a third of total marrow iron uptake. With increasing erythropoiesis, the increase in iron uptake in expansion areas was less marked than the increase in the central areas. Erythropoiesis in expansion areas was usually not of major quantitative importance but could nevertheless reach the erythropoiesis of a normal adult.

Minor myeloid component in Ph chromosome-positive acute lymphoblastic leukaemia: correlation with cytogenetic pattern and implication for poor response to therapy

Morphological, immunological and cytogenetic features were studied in 27 adults presenting with Ph chromosome‐positive acute lymphoblastic leukaemia (ALL), in correlation with clinical outcome.

The spleen and haemolysis: evaluation of the intrasplenic transit time.

The mean intrasplenic red cell transit time (STT) and the slow mixing splenic red cell volume (SSV) have been measured in patients with hereditary spherocytosis (HS), autoimmune haemolytic anaemia (AIHA) and lymphoproliferative disease (LD). There was an inverse relationship between the mean red cell life span (MRCLS) and the STT in HS (r=–0·96, P<0·001) and in AIHA (r=–0·90, P<0·001). No such relationship existed in LD. The size of the spleen and the SSV were not related to the severity of haemolysis. Our data offer strong evidence for the conditioning effect of the spleen on HS‐ and AIHA red cells and suggest that the STT is an index of the adverse effect of the spleen on red cells in patients with HS or AIHA.

Assessment of the Sites of Red-cell Destruction Using Quantitative Measurements of Splenic and Hepatic Red-cell Destruction

Red cell survival, surface counting indices, the splenic and hepatic contribution to red cell destruction and the rate of splenic and hepatic red cell destruction were measured in 29 patients. Splenectomy was performed in 14. No correlation could be found between the splenic excess count index and both the amount and rate of red cell destruction in the spleen, but the rate of splenic and hepatic red cell destruction was related to the rate of disappearance of red cells from the circulation. The mean fractions of red cell destruction in spleen and liver were 46.1%± 20.5 (SD) and 11.7%± 4.2 (SD) respectively. After splenectomy, the haematocrit returned to normal in all patients despite fractions of red cell destruction in the spleen not exceeding 60%. Although the measurements of the splenic red cell destruction rate and of the fraction of red cell destruction in the spleen provide more precise information on the role of the spleen in red cell destruction, their prognostic value in patients who underwent splenectomy was not obvious.

Imipenem Cilastatin Versus Piperacillin Plus Amikacin As Empiric Therapy in the Treatment of Febrile Episodes in Neutropenic Patients With Hematologic Malignancies

Recently, new beta-lactam antibiotics, such as imipenem/cilastatin (IMP) with an unusually broad antibacterial spectrum and especially an adequate P. aeruginosa activity, have introduced the possibility of using prospective agent as empiric management of febrile granulocytopenic patients. We randomized 83 febrile neutropenic cancer patients for a prospective evaluation of two regimens: IMP versus piperacillin plus amikacin (PA). Both patients groups were similarly distributed with regard to age, sex, primary diagnosis and degree of granulocytopenia. More than 20% of the 74 evaluable patients had bacteraemia. The overall response rate for clinically or microbiologically documented infections was 90% in the IMP regimen versus 76% in the PA regimen, but statistical difference was not achieved. All bacteraemias in the IMP group but only 60% in the PA group were cured, however statistical difference was not achieved. IMP had to be discontinued in only one patient and the most common side effects were nausea and vomiting; no seizures were noted. Nephro- and ototoxicity, skin rash and bleeding have been the major side effects requiring drug discontinuation in 6 patients treated by PA. In conclusion, these data suggest that IMP used alone is safe and as effective as the combination of P plus A for the management of febrile granulocytopenic patients with haematologic malignancies. Further studies on a larger number of patients are needed to confirm these findings.

Le Syndrome Hypereosinophilique (She) A Propos De Deux Cas Et Revue De La Litterature.

SummaryIdiopathic hypereosinophilic syndrome is characterized by prolonged eosinophiliaof undetected cause and multiple organ system involvement (lung, kidney, nervous system, skin, …). Nevertheless, the prognosis has been correlated with heart involvement, which usually results in a restrictive cardiomyopathy with apical obliteration by fibrosis, mural thrombi and mitral and tricuspid regurgitation.This disease has a wide range of severity: some patients suffer from a real myeloproliferative syndrome and may develop blastic transformation while others present only skin involvement or are asymptomatic.Corticosteroids and hydroxyurea are both effective treatments. Interferon a seems to be active for the myeloproliferative form of the disease.Cytotoxic activity of activated eosinophil granular proteins may play an important role in tissue damage. The cause of eosinophilic proliferation (primitive malignant proliferation or resulting from a T lymphocyte stimulus) and activation remains uncertain.