[Refractory anaemia with ringed sideroblasts (RARS) associated with marked thrombocytosis: a provisional entity in the WHO classification of haematological malignancies]. / Thrombocytose majeure et anémie réfractaire avec sidéroblastes en couronne: une entité provisoire dans la classification OMS des hémopathies.

The WHO classification describes a group of myelodysplastic/myeloproliferative diseases, including a provisional entity, refractory anaemia with ringed sideroblasts (RARS) associated with marked thrombocytosis, underlining that is a provisional entity without consensus of belonging to myelodysplastic rather than to myeloproliferative syndromes. The authors report two cases with features of refractory anaemia with excess of ringed sideroblasts and marked thrombocytosis. In the first case, RARS is concomitant with thrombocytosis and fits the WHO criteria for this temporary entity. The second case is a typical RARS, who developed a thrombocytosis after several years and emphasizes that a link, at least progressive, exists between RARS and myeloproliferative disorders. The authors summed up the various situations related to secondary or primary acquired sideroblastic anaemia, likewise to primitive and reactive thrombocytosis. The cases of RARS + marked thrombocytosis reported in the literature are few in number and do not allow to settle between a particular form of myelodysplastic syndrome and a myeloproliferative disorder, a fully justified reason to classify these patients in a temporary group. To date, there is no codified therapy for this disorders.

rHuEpo before high-dose therapy allows autologous peripheral stem-cell transplantation without red blood cell transfusion: a pilot study.

To decrease red blood cell (RBC) transfusion requirements during high-dose therapy (HDT) for hematological malignancies, we conducted a pilot study to assess the effect of recombinant human erythropoietin (rHuEpo) given during chemotherapy before HDT and autologous peripheral stem-cell transplantation (APSCT). The transfusion histories of 15 HDT and APSCT for hematological disease performed in 11 consecutive patients who received rHuEpo (10 000 U subcutaneously three times/week) were compared to those of 22 HDT and ASCT performed in 17 consecutive historical controls matched for hematological parameters. rHuEpo increased the hemoglobin (Hb) level from 10.3+/-2.3 g/dl at diagnosis to 12.9+/-2.2 g/dl at the time of HDT in 11 patients; no major adverse effects occurred. Compared to historical controls (95%, 21/22), RBC transfusion requirements were significantly lower for rHuEpo recipients (26%, 4/15) (P=0.00001) and rHuEpo responders (15%, 2/13) (P=0.000002). After HDT and APSCT, fewer RBC transfusions were needed: 3.3, 1.2 and 0.3 RBC units for controls, rHuEpo recipients and rHuEpo responders, respectively (P=0.006 and 0.00002). Therefore, rHuEpo should be administered before, and not after HDT and APSCT, to lower RBC transfusion requirements after HDT and APSCT.

[Asplenia in the Hodgkin's patient]. / L'asplénisme du patient hodgkinien.

FEWER INDICATIONS AFTER SPLENECTOMY: Real therapeutic progress has been achieved over the last fifty years for patients with Hodgkin's disease known for their chronic immunodepression. Since the advent of effective chemotherapy protocols such as ABVD, and more recently intensive chemotherapy completed as needed with an autograft, splenectomy is no longer performed for therapeutic purposes but may be indicated for its contribution to diagnosis. STRATIFICATION OF RISK OF ASPLENISM: There remain however several questions concerning the infectious complications in these patients given chemotherapy and splenic radiotherapy. One of the objectives of this work was to propose a stratification of risk of asplenism as a function of treatments administered, the level of initial immunodepression, and the age of the patient.

[Immunohistochemical characterization of acute leukemia. Study of 31 bone marrow biopsies]. / Caractérisation immunohistochimique des leucémies aiguës. Etude de 31 biopsies ostéo-médullaires.

An immunohistochemical study by avidin-biotin-peroxidase was performed on paraffin-embedded and decalcified bone marrow biopsies in 31 acute leukemias (19 myeloid and 12 lymphoblastic). The Ulex Europaeus lectin and 14 antibodies (anti-CD45, -CD34, -myeloperoxidase, -lysozyme, -CD15, -CD68, -carcinoembryonic antigen, -factor VIII-related antigen, BNH9, anti-CD45RO, -CD3, -CD20, DBB42 and DBA44) were tested. All acute myeloid leukemias from M0 to M5 type were stained by either the anti-myeloperoxidase or anti-lysozyme antibodies. CD68, CD15 and the carcinoembryonic antigen were respectively expressed in 80%, 40% and 20% of myeloid leukemias from M1 to M5 type. The Ulex Europaeus lectin and the anti-factor VIII-related antigen antibody stained only the M7 leukemia and the anti-CD3 antibody stained only the T acute lymphoblastic leukemia. DBB42 was expressed by 63% of B-lineage lymphoblastic leukemias and CD20 by 36%. No leukemia was stained by DBA44. Immunohistochemistry on bone marrow biopsy can assess the lineage of most acute leukemias with the use of a panel of antibodies such as the anti-myeloperoxidase, -lysozyme, -CD68, -CD20, DBB42, -CD3, BNH9, anti-factor VIII-related antigen antibodies and the Ulex Europaeus lectin.

[Histopathologic lesions in erythromelalgia during essential thrombocythemia]. / Lésions histopathologiques de l'érythromélalgie au cours d'une thrombocytémie essentielle.

Erythromelalgia is a vascular disorder of the extremities and is sometimes related to myeloproliferative syndrome with thrombocythemia. We report the cutaneous histopathology in case of erythromelalgia that revealed a thrombocythemia vera. Small arteries were occluded by thrombi of different age and narrowing of the lumen occurred by intimal proliferation of smooth muscle cells. There was no involvement of venules or capillaries. These vascular changes are highly suggestive of erythromelalgia and have not to be confused with necrotizing and/or granulomatous angiitis because of absence of fibrinoid necrosis and sparse inflammatory cells.

Ankylosing spondylitis and monoclonal gammopathies.

From 1960 to 1990, 557 patients with ankylosing spondylitis (428 men, 129 women) were diagnosed and indexed in the department of rheumatology. Monoclonal gammopathies were found in seven (five men, two women) patients (1.3%). With one exception, ankylosing spondylitis preceded monoclonal gammopathies by many years. The distribution of the isotypes of the mIg found in these seven patients was striking when compared either with previous reports of an association between ankylosing spondylitis and monoclonal gammopathies or with local data on the epidemiology of monoclonal gammopathies: five patients with IgG, four of them of the lambda (lambda) type, and two IgM, both of the kappa (kappa) type were found; no patients with mIgA were recorded. Two patients were HLA-B27 positive and had slight and transient monoclonal gammopathies, whereas three subjects were HLA-B27 negative and had important spikes, corresponding in two subjects to malignant diseases. This observation raises the question of whether the coexistence of HLA-B27 and ankylosing spondylitis might provide a protective action. Epidemiological studies are required to clarify such points.

Determination of anti-neutrophil cytoplasm antibodies (ANCA) specificity by immunofluorescence on chronic myelocytic leukemia cells.

ANCA positive sera, detected by the standard immunofluorescence method, derived from 37 patients with vasculitis were studied using formalin-acetone fixed chronic myelocytic leukemia cells (CML). All 37 sera were positive on CML cell smears. Furthermore formalin-actone fixation selectively impaired antinuclear antibody binding without reducing ANCA staining and thus facilitated differentiation of these autoantibodies which is often difficult with the standard immunofluorescence method. Two unequivocal and mutually exclusive ANCA binding patterns were identified using the CML smears: (1) type I with diffuse granular binding confined to the polymorphonuclear (PMN) cell lineage and preferentially staining immature cells; (2) type II with similar binding to the PMN cell lineage and, in addition, granular staining of the basophils. All type I antibodies were associated with a c-ANCA pattern suggesting that the major antigen recognized by these antibodies, recently identified as proteinase 3, is not detectable in basophils. The type II pattern was detected in both p-ANCA (84%) and c-ANCA (16%) positive sera. The type I sera remained positive on PMN cells from a myeloperoxidase (MPO) deficient subject and anti-MPO antibodies could not be detected in this group by ELISA. Conversely the type II pattern occurred in the presence of anti-MPO antibodies identified by immunofluorescence, ELISA and dot-blot with the exception of a single serum with antilactoferrin antibody. Type I binding only was observed in Wegener's granulomatosis (WG) but both patterns were found in microscopic polyarteritis (MPA) and rapidly progressive glomerulonephritis (RPGN).

Splenic lymphoma with circulating villous lymphocytes. Report of a case with immunologic and ultrastructural studies.

A case of splenic lymphoma with circulating villous lymphocytes is reported. Short surface cellular expansions were observed on blood and marrow films and by transmission electron microscopy. The immunophenotype was that of mature B cells without CD5, CD10, CD11c, or CD25 expression or tartrate-resistant acid phosphatase. Despite a basophilic plasmacytoid-like cytoplasm, this case of splenic lymphoma with circulating villous lymphocytes differed from splenic immunocytoma in that immunofluorescence and ultrastructure suggested that the neoplastic cells did not possess high levels of intracytoplasmic immunoglobulin. Treatment of cytopenia was best achieved by splenectomy and the total follow-up thus far (30 months) seems to indicate a case of low-grade malignant lymphoma.

Home treatment of febrile neutropenia: an empirical oral antibiotic regimen.

Between May 1988 and November 1989, 68 consecutive febrile courses supervening after polychemotherapy for lymphoma outpatients (median age 50 years) were treated by the combination of oral Pefloxacin/Amoxicillin Clavulanic acid. In terms of median data, neutropenia appeared on d9 [d1-d17], and lasted 5 days [2-9] with a PMN nadir observed at 0.104 x 10(9) [0-0.5 x 10(9)/l], while fever rose on d10 [1-24]. In 59 cases (87%), fever and/or focal symptoms disappeared within 3 days, after which treatment was maintained for 7 days. Nine failures were observed, of which 2 were due to abandonment of treatment, 2 to vomiting and 5 to persistence of the original symptoms. Meti Susceptible-Staphylococci were found in blood samples from 2 patients, one of whom, with grade IV lymphoma that had proved resistant to chemotherapy, died. The treatment was found to be effective and well tolerated, offering a good alternative to hospitalization during a transient chemotherapy-induced neutropenia.

Acute Myeloid Leukemia in Children Less Than Two Years Old: Clinical, Cytologic and Cytogenetic Correlations Comparison with other age categories. A clinical survey of 39 cases.

Thirty-nine children under two years of age with newly diagnosed Acute Myeloid Leukemia (AML) were consecutively admitted to the Hôpital Saint-Louis (Paris) over a ten-year period (1978-1987). Nineteen were under one year of age at diagnosis and 3 had congenital leukemia. Comparison of FAB sub-classes among the different age categories showed that AML with a monocytic component (M4 and M5) (44%), and AML with megakaryocytic elements (M7) (21%), were much more frequent in children before the age of two years. This comparison was made on a series of 1409 AML and 859 ALL diagnosed in the same hospital during a twelve years period of time. During this period of observation, the compared incidence of ALL and AML showed 50% for each category for children under 2 years, 73% ALL versus 27% AML for children under 15 years, and 18% ALL versus 82% AML for adults. Of the 29 cases subjected to cytogenetic study, 62% had clonal chromosomal abnormalities, the most frequent being a translocation involving the long arm of chromosome 11. With a 72 months median follow-up for patients who are still alive, the 6-year actuarial survival was 23%, while the event free interval for Complete Remission (CR) patients was 35%. For the M4-M5 subgroup, these results were 37% and 50% respectively with a plateau reached at 27 months. M4-M5 FAB types represented the most common leukemia at this age, and these usually correlated with translocation of the long arm of chromosome 11. We obtained an unexpectedly high rate of survival, with acceptable sequellae, for children treated with intensive chemotherapy.

Transient acanthosis nigricans following bone marrow transplantation for lymphoid malignancy.

Transient acanthosis nigricans (AN) was observed 3 months after bone marrow transplantation (BMT) for lymphoblastic lymphoma. The patient had no endocrine abnormality and had not received any drug known to induce AN. Forty-eight months post-BMT no malignancy recurred or appeared. Although usual hyperkeratosis, papillomatosis and acanthosis were present on skin biopsy, these were associated with the finding of keratinocyte necrosis and CD8+ lymphocytic infiltrate. It is suggested that graft-versus-host disease may be one of the triggers for AN.