Antoine Broustet

Peripheral neuropathies and lymphoma without monoclonal gammopathy: a new classification.

SummaryRecent progress in immunopathological studies of peripheral nerve and lymph node fragments together with 16 personal cases and numerous clinicopathological reports have suggested a new classification of peripheral neuropathies (PN) and lymphomas. These are: (1) PN due to local infiltrations by a T-cell lymphoma; (2) acute polyradiculoneuritis due to active demyelination and associated with infiltrates of a T-cell lymphoma in the epineurium, resembling Mareks disease (which is a T-cell lymphoma); (3) B-cell lymphoma proliferation which may be restricted to or predominate in the peripheral nervous system, with a large clinicopathological heterogeneity ranging from localized forms to ascending polyradiculoneuropathies; (4) angiotropic lymphoma, which is a B-cell lymphoma and may present as an acute mononeuropathy; (5) patients with acquired immunodeficiency syndrome due to lymphomatous infiltrates in the endoneurium, of which 2 cases of PN have been reported; (6) PN associated with organomegaly, endocrinopathy, M-component and skin lesions, certain cases being associated with a plasmocytoma and sometimes Castlemans disease but without any monoclonal gammopathy; (7) classic Guillain-Barré syndrome, prone to develop in patients with extraneural lymphoma but without any lymphomatous infiltrates in the peripheral nervous system; (8) certain cases (4 out of 16 in our series) where there is no clear relationship between PN and lymphoma, and there are mainly features of axonal degeneration. Inflammatory perivascular infiltrates were sometimes present in the epineurium.

Vascularites leucocytoclasiques et hémopathies malignes (12 observations)

We report 12 cases of leucocytoclastic vasculities associated with myelocytic (7 cases) or lymphocytic (5 cases) blood diseases. The clinical features, laboratory abnormalities and pathological findings are presented. In all cases a drug-induced and/or infective origin could be ruled out, and patients with cryoglobulinaemia were excluded. As in other cases found in the literature, in some patients vasculitis was present several weeks or months before the blood disease was discovered, while in others both conditions developed simultaneously or vasculitis appeared in the course of the blood disease. None of these three possibilities seemed to make the prognosis worse. The pathogenesis of the vasculitis-malignant blood disease association is uncertain. Immune complexes of tumoral origin undoubtedly have some responsibility, but some deficiency of phagocytic cells or chemotactic factors also play a role. Cases such as ours enable the significance of vasculitis to be understood when it occurs in a patient with malignant blood disease, but above all they should prompt investigations for malignant blood disease in patients with cutaneous and/or systemic vasculitis, as is being done in patients with pyoderma gangrenosum or Sweets syndrome.

Prognostic value of age and bone marrow karyotype in 78 adults with acute myelogenous leukemia

Bone marrow karyotypes of 78 adult patients with acute myelogenous leukemia (AML) were studied at the time of diagnosis, with special reference to the Sakurai and Sandberg classification (NN, karyotype completely normal; AN, mixture of normal and abnormal metaphases in the karyotype; AA, totally abnormal karyotype). The results showed no difference in complete remission rate (CR) or in survival time between the NN and the AN groups, but highly significant differences between the AA group and the NN and AN groups (whether taken together or separately). When studying the relationship between age and survival time, we found that 59 years of age was a frontier between two homogeneous groups having quite different prognoses. The NN/AN/AA classification had a good prognostic value in patients under 59 years of age. In older patients, no correlation was found between the classification and complete remission rate or survival time.

Acute myeloid leukemia with marrow hypereosinophilia and chromosome 16 abnormality

This article reports six cases of acute nonlymphocytic leukemia (ANLL) and an abnormal chromosome #16. All had the same hematologic pattern at diagnosis, i.e., peripheral blood hyperleukocytosis with a high percentage of monocytes and blast cells. The bone marrow showed three different cell populations: (a) myeloblasts, (b) monocytes and promonocytes, and (c) abnormal eosinophils. In three cases, an ultrastructural study confirmed the cytologic data. In all six cases, the diagnosis was acute myelomonocytic leukemia with bone marrow eosinophilia (M4-Eo). All cases showed an abnormal chromosome #16 in the bone marrow cells; in four cases, well-banded chromosomes were obtained, showing a pericentric inversion inv(16)(p13;q22). One patient had a 4-year remission, and another is still in remission 14 months after diagnosis. Three patients relapsed 7, 9, and 20 months after diagnosis. The last patient died soon after diagnosis. Thus, we do not support the hypothesis that patients with M4-Eo ANLL and chromosome #16 abnormality have a favorable prognosis.

Megakaryoblastic Transformation of Primary Thrombocythemia

The progression of primary thrombocythemia (PT) into acute leukemia was diagnosed in a 64-year-old man who was previously treated by hydroxyurea without alkylating agents or radioactive phosphorus. Such an event has only been reported in very rare cases. The blast cells had a lymphocytic morphology but were identified as promegakaryoblasts by the ultrastructural demonstration of platelet peroxidase. These data suggest that a megakaryoblastic transformation could occur in PT as had previously been reported in chronic granulocytic leukemia.

Autologous blood stem cell grafting in hematological malignancies. Present status and future directions.

Abstract It has been demonstrated that peripheral blood stem cells (PBSC) are able to reconstitute hematopoiesis as well as bone marrow cells. This review discusses the possible advantages of PBSC transplantation over autologous bone marrow transplantation (ABMT) including both the kinetics of hematopoietic reconstitution and the outcome of the patients following transplantation. The main advantage of PBSC transplantation is the very short aplastic phase observed after transplantation leading to a decrease of morbidity or even mortality. However, the administration of hematopoietic growth factors after bone marrow transplantation is followed by a reduction in the length of aplasia similar to that expected after PBSC transplantation. In patients with hematological malignancies, the risk of relapse seems to be equivalent after either PBSC transplantation or ABMT. Thus, the future place of PBSC transplantation must be further investigated.

Acute erythroblastic leukemia presenting as acute undifferentiated leukemia: A report of two cases with ultrastructural features

This report describes two elderly patients with acute leukemia in which blast cells were undifferentiated with conventional light microscopy (L.M.) and cytochemistry. Blast cells were identified as belonging to the erythroblastic line by their ultrastructural features: glycogen deposits, lipidic vacuoles, cytoplasmic ferritin molecules and rhopheocytotic invagination. Moreover, blast cells were surrounding a central macrophage. Thus, these two patients had acute erythroblastic leukemia which differs from erythroleukemia (M6 of FAB classification) in which blast cells present myeloblastic characteristics.

Associated abnormalities of chromosomes 1, 5, and 11 in dysmyelopoietic syndromes

Two patients with dysmyelopoietic syndrome presented the same cytogenetic pattern in their bone marrow cells, i.e., trisomy of chromosomes #1 and #11, and terminal deletion of chromosome #5 (q13-q14). Two similar cases have been described in the literature. It is suggested that this cytogenetic pattern could be a nonrandom event.

Hydroxyurea versus interferon alfa-2b in chronic myelogenous leukaemia: Preliminary results of an open French multicentre randomized study

In order to compare the effects of interferon versus hydroxyurea for the treatment of chronic myelogenous leukaemia (CML), 58 CML patients, having received no previous treatment, were randomized into two treatment groups (hydroxyurea or interferon) for an open multicentre study from 1 May 1987 until 1 July 1990. Fifty patients were evaluable: 24 in the interferon group and 26 in the hydroxyurea group. Haematological response was obtained in 16/24 interferon-treated patients and 23/26 hydroxyurea patients. Failure to obtain haematological remissions occurred in eight of 24 interferon-treated patients and in three of 26 hydroxyurea patients. Four interferon-treated patient failures and one hydroxyurea-treated failure were due to drug intolerance. Progression occurred in one interferon-treated patient and in three patients given hydroxyurea. Fourteen of 16 patients in the interferon group and 17/23 in the hydroxyurea group continue on study and show no progression.

The presence of particles resembling human T-cell leukemia virus type I at ultrastructural examination of lymphomatous cells in a case of T-cell leukemia/lymphoma

Background. Cases of adult T‐cell leukemia/lymphoma (ATLL) resulting from human T‐cell leukemia virus type I (HTLV‐I) have been observed mainly in the southern part of Japan. Recently, the authors performed a second examination of cutaneous, muscle, and nerve biopsy specimens from a French white woman who died of ATLL in 1979.