Cigarette smoking-related persistent polyclonal B lymphocytosis. A premalignant state.

A case of cigarette smoking-related polyclonal B lymphocytosis, with binucleated cells, was observed. The condition was persistent for two years. We indicated the abnormal ultrastructural features and suggest that this unexplained syndrome could be a premalignant state.

[Systemic mastocytosis and malignant mastocytosis]. / Mastocytoses systémiques et mastocytoses malignes.

Systemic mastocytosis is a rare, often undiagnosed disease characterized by the proliferation of mast cells in several tissues. The clinical symptoms are related to the mast cell infiltrates, but also to the release of numerous mediators. Malignant mastocytosis is a term that refers to two different entities: aggressive mastocytosis, where the outcome with a myeloproliferative disease, where the latter governs the prognosis. The potential severity of these diseases justifies the development of more intensive therapeutic measures.

[Automation for the evaluation of blood basophilia]. / Apport de l'automatisation à l'appréciation de la basocytose sanguine.

We report here the first results of our experience in evaluating the blood basophils count through the "H 6000" automatic counter. An artefact is described through which this count may falsely be increased. The value of basophil cells rates in some diseases is being discussed.

Questions concerning plasma cell leukaemia.

The classical concept of plasma cell leukaemia (PCL) is reviewed. This disease invariably concerns myeloma, either in its terminal phase (secondary PCL) or particularly rapid and aggressive (primary PCL). A more precise definition of primary PCL would be given by the term leukaemic myelomatosis. Characterization of the membrane antigen CD 56, responsible for the cytoadhesion of malignant plasmocytes in the bone marrow, today offers a convincing pathogenic hypothesis to elucidate this affection which would now appear to be no more than a variant form of myeloma. Acute immunoblastic-plasmoblastic leukaemia and mature cell leukaemic lymphoma would seem to be pathologically related to PCL. Finally, the problems posed by the "enigmatic" plasmacytoid monocyte are discussed.

[Autoimmune hemolytic anemia anemia followed by acute granulocytic leukemia (author's transl)]. / Anémie hémolytique à auto-anticorps suivie d'une leucémie myéloïde aiguë.

The authors report a case of acute myeloid leukemia developping 5 years after the onset of an autoimmune hemolytic anemia. The cytological origin of the proliferation is difficult to assess: the presence of prominent membranous complexes and a strong positivity of acid phosphatase reaction favour its megacaryocytic origin. However the cytological evolution pleads for the development of successive clones of monocytic origin, may be modified by the treatment. Are the two diseases successive consequences of preexisting state of immunodepression or was such a state created by the autoimmune anemia?

[Unusual giant inclusions in blast cells during acute transformation of chronic myeloid leukemia. Cytochemical and ultrastructural study (author's transl)]. / Inclusions anormales dans les blastes b'une transformation aiguë de leucémie myéloïde chronique. Etude cytologique et ultrastructurale.

Unusual giant inclusions are reported in the blast cells during the blastic crisis of a chronic granulocytic leukemia. These inclusions coudl have their origin in degenerated mitochondria, and acid phosphatase positivity suggests fusion with lysosomes. A possible viral etiology is discussed.

[Autoimmune hemolytic anemia followed by acute granulocytic leukemia (author's transl)]. / Anémie hémolytique à auto-anticorps suivie d'une leucémie myéloïde aiguë.

The authors report a case of acute myeloid leukemia developing 5 years after the onset of an autoimmune hemolytic anemia. The cytological origin of the proliferation is difficult to assess: the presence of prominent membranous complexes and a strong positivity of acid phosphatase reaction favour its megacaryocytic origin. However the cytological evolution pleads for the development of successive clones of monocytic origin, may be modified by the treatment. Are the two diseases successive consequences of preexisting state of immunodepression or was such a state created by the autoimmune anemia?

[Tubular inclusions in the lymphocytes in a case of "hairy cells leukemia" (author's transl)]. / Inclusions tubulaires dans les lymphocytes d'une leucémie a tricholeucocytes

A tubular inclusion (Hovig type) was found in the cytoplasm of the blood lymphocytes of a case of hairy cells leukemia. Its cytochemical and ultrastructural features are described ; its signification remains unknown.

[Pure megaloblastic refractory anemia. Prolonged complete remission following androgenotherapy]. / Anémie réfractaire pure mégaloblastique. Rémission complète prolongée après androgénothérapie.

In a case of pure megaloblastic refractory anemia, a complete remission is reported. This remission has been obtained by prolonged high dose androgenotherapy and has now lasted for more than 5 years. The practical therapeutic consequences are discussed.

[Coexistence of myeloma and chronic myeloid leukemia]. / Coexistence d'un myélome et d'une leucémie myéloïde chronique.

The case of an eighty-one-year-old woman who had myeloma followed 18 months later by chronic myeloid leukemia with Philadelphia chromosome unrelated to therapeutic interferences is reported. This case is compared to other reports of lymphoid hemopathies associated with myeloid hemopathies found in the medical literature. The different etiopathogenic hypotheses are discussed, particularly in the light of recent studies of chromosomes. Such caryotypic studies in malignant hemopathies may improve our knowledge of the connexions between apparently dissimilar diseases and prove helpful for the understanding of cell differentiation from pathological data.

Plasma cell leukaemia mimicking acute monocytic leukaemia in the course of multiple myeloma.

We report a case of acute leukaemia occurring early in the course of IgA multiple myeloma. Ultrastructural studies, immunophenotyping and karyotyping were required to identify the origin and clonality of the leukaemic cells. Although ultrastructural examination of the blast cells revealed both monocytoid and plasma cell features, all cells expressed the CD 38 antigen and intracytoplasmic kappa light chains, while karyotyping revealed a clone with numerous abnormalities, leading to the diagnosis of clonal plasma cell disease. The occurrence of leukaemia in multiple myeloma is discussed.

[Peroxidase deficiency in eosinophils: an optical and electron microscopy cytologic study apropos of a case]. / Déficit en peroxydase des éosinophiles: étude cytologique en microscopie optique et électronique à propos d'un cas.

A case of eosinophil peroxidase deficiency (EPO), discovered using an automated differential leukocyte cell counter, was studied using optical and ultrastructural cytologic methods. The blood and bone marrow eosinophilic cells had an abnormal ultrastructural aspect with important modifications of the specific granule matrix. No peroxidase activity could be revealed in these cells using optical or ultrastructural techniques. The mechanism and significance of this anomaly raise the question of the physiological function of this enzyme.

Activated partial thromboplastin time performed on capillary blood.

Activated partial thromboplastin time is one of the most frequently used assay in haemostasis investigation, but sampling of venous blood is often difficult in newborns (as well as some adult patients). We analysed a method described by Zondag et al [9] performed on capillary blood samples. We studied normal adults and newborns, patients with liver diseases, and those receiving therapy with vitamin K antagonists and heparin. Capillary assay was correlated with venous blood in normal subjects, in patients with liver diseases and during therapy by vitamin K antagonists. However results both in newborns and adults during heparin therapy were not accurate.

[Chronic lymphoid leukemia followed by acute monocytic leukemia]. / Leucémie lymphoïde chronique suivie d'une leucémie aiguë monocytaire. Etude clinique et cytologique.

A 62 year old patient with CLL treated by alkylating drugs for more than three years, underwent acute leukaemia (AL). The clinical particularities of the onset and course of this AL, the cytological, cytoenzymological and ultrastructural characteristics of the blasts are discussed in order to find a relation between the two hemopathies. A possible drug-induced etiology of this AL is proposed.

[Hereditary deficiency of factor XI in a Portuguese family, the propositus of which had Behçet's disease (author's transl)]. / Déficit constitutionnel en facteur XI: description chez une famille Portugaise dont le propositus a une maladie de Behçet.

The finding of a hereditary deficiency of factor XI in a Portuguese family confirms that this abnormality is not confined to Ashkenazi Jews. Its transmission is autosomal, recessive and of variable expression, and in this particular family it appeared to be asymptomatic. When infused post-operatively in fresh frozen plasma, Factor XI has a half-life of about 24 hours and a recovery rate of almost 100%. The propositus, a 57-year-old woman, appears to have been "protected" by a homozygous deficiency of Factor XI against the thromboembolic complications of a Behçets disease which had been present for 30 years. Plasma exchange and perfusions of fresh frozen plasma were probably responsible for the regression observed in the symptoms (particularly ocular symptoms) of the disease, then in active phase.

[An essential hypereosinophilic syndrome. Cytological, cyto-enzymological and ultrastructural studies (author's transl)]. / Syndrome d'hyperéosinophilie essentielle : un cas avec études cytochimique, cytoenzymologique et ultrastructurale des ésoniophiles.

A new essential hypereosinophilic syndrome has been reported in a 53 years old man who presented an important blood and bone marrow hypereosinophilia of unknown etiology since 5 years. The evolution of the clinical course included neurological accidents (hemiplegia, spasmodic quadriplegia, seizures) congestive heart failure, hepatomegaly, transient renal involvement, pulmonary infiltrates with pleural hemorrhage, episodes of diarrhea and fever with weight loss. The cytological study of the eosinophiles showed the existence of abnormal inclusions which were confirmed by the electron microscope. These and cytoenzymological abnormalities led to discussion of the relationship between the essential hypereosinophilic syndrome and eosinophilic leukemia.

[Testing the hemalog D in a hematology department of a general hospital in Paris (author's transl)]. / Expérimentation de l'hemalog D dans un laboratoire de l'assistance publique de Paris.

Europe's firs Hemalog D was installed in the Hematology Laboratory of the Franco-Musulman Hospital at Bobigny, just outside Paris, in March 1975. The authors' experience with the apparatus since that date has enabled them to analyze the significance of "alarms", "high peroxidase", "large unstained cells", "remainder" and "low rate" in patients with and without hematologic disorders. On the basis of these results it has been possible to define the fate of the various blood cells in the Hemalog D, the role of the apparatus in the ivestigation of hematologic disorders and the type of "cooperation" between the hematologist and the Hemalog D.