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Résumé: La constatation d'une monocytose est un phénomène fréquent, qui nécessite confirmation par lecture au microscope par un biologiste aguerri, permettant de s'affranchir des pièges cytologiques classiques comme la présence de tricholeucocytes, de cellules de Sézary ou de blastes monocytaires. Dans la grande majorité des cas l'origine réactionnelle est très facilement mise en évidence par le contexte et/ou la présence d'un syndrome inflammatoire biologique. Plus rarement le diagnostic est orienté vers une pathologie à éosinophiles ou une leucémie aiguë. Dans les autres cas, leucémie myélomonocytaire chronique, néoplasies myéloprolifératives ou syndromes myélodysplasiques avec monocytose pourront être évoquées. En l'absence d'élément diagnostique de certitude et la présence de formes « frontières » le diagnostic différentiel entre ces 3 entités n'est pas toujours simple, nécessitant, selon l'Organisation mondiale de la santé (OMS) investigations moléculaires et élimination de toute cause réactionnelle de monocytose. Bien que les études anatomo-pathologiques et phénotypiques par cytométrie en flux ne soient actuellement pas recommandées par l'OMS, ces investigations ont probablement un intérêt dans l'évaluation des cas difficiles. The discovery of a monocytosis is a frequent phenomenon, requiring confirmation by reading under a microscope by an experimented biologist, to overcome usual cytological traps such as the presence of hairy cells, promonocytes or monoblasts. In the vast majority of cases the secondary origin is very easily found by the context and/or the presence of a biological inflammatory syndrome. More rarely the diagnosis is directed towards an eosinophilic pathology or an acute leukemia. In other cases, CMML, MPN or MDS with monocytosis may be highlighted. In the absence of any pathognomonic element and the presence of "borderline" forms the differential diagnosis between these 3 entities is not always straightforward, requiring, according to WHO, molecular investigations and elimination of any reactive cause of monocytosis. Although histological, immunohistochemical and phenotypic flow cytometric studies are not currently recommended by WHO, these investigations could be of interest in the evaluation of difficult cases. [ABSTRACT FROM AUTHOR]

Résumé: Le bilan étiologique d'une anémie hémolytique est souvent simple et standardisé permettant de retrouver le plus souvent une origine immune, infectieuse, médicamenteuse, une microangiopathie thrombotique (MAT) ou une pathologie constitutionnelle par anomalie membranaire, enzymatique ou de l'hémoglobine. Parfois aucune cause n'est retrouvée. Les résultats du bilan biologique initial nécessitent parfois une interprétation délicate dont la méconnaissance peut amener des erreurs dans le diagnostic d'orientation, source d'explorations inutiles. Le but de cet article est de rappeler les nuances dans l'interprétation de certains résultats et de pointer du doigt quelques étiologies d'anémies hémolytiques constitutionnelles ou acquises plus rares, souvent méconnues, qu'il faut savoir rechercher en cas d'impasse diagnostique. The most frequent causes of hemolytic anemias are immune or infectious diseases, drug induced hemolysis, thrombotic microangiopathies, hereditary spherocytosis, glucose-6-phosphate dehydrogenase or pyruvate kinase deficiencies, thalassemia's and sickle cell disease. Sometimes no cause is found because a rarer etiology is involved. The goal of this review is to remember some unfrequent constitutional or acquired causes and to point out difficulties to avoid wrong interpretations of analysis results. [ABSTRACT FROM AUTHOR]

Résumé: La neutropénie, caractérisée par une concentration sanguine en polynucléaires neutrophiles inférieure à 1,5 G/L, est un motif assez fréquent de consultation en médecine interne. Les formes aiguës sont principalement dues à une prise de médicaments ou à une infection. Les neutropénies chroniques, définies habituellement par une persistance au-delà de 3 mois, peuvent être en rapport avec de nombreuses étiologies dont l'exploration nécessite un cheminement précis. Cet article, outre le rappel des critères à respecter pour engager une exploration, décrit les principales causes des neutropénies de l'adulte, en proposant un algorithme pouvant être utilisé en pratique courante. Adult neutropenia, defined as a blood neutrophil count below 1.5 G/L, is a common condition. The most common cause of acute neutropenia is a drug-related reaction or an acute infectious disease. In chronic forms many etiologies are possible, sometimes poorly-defined, requiring precise explorations. The purpose of this article is to recall the required criteria for exploring neutropenia and to point out the algorithm of explorations in order to find the cause. Etiologies of acute and chronic forms are also detailed. [ABSTRACT FROM AUTHOR]

Although two clusters have been identified in France, constitutional factor XI deficiency is a rare disorder. Acquired factor XI deficiency is extremely rare. The management of factor XI deficiency is not staightforward because of the unpredictable bleeding tendency that does not clearly relate to the factor XI level. Other haemostastis parameters have to be taken into account to evaluate the bleeding tendency. We report the cases of a congenital factor XI deficiency, an acquired factor XI deficiency and a von Willebrand disease associated to a factor XI deficiency. On the other hand, some interferences can lead to underestimation of factor XI and we report the case of an interference by lupus anticoagulant. The objective of this review is to better understand how to manage a reduced factor XI level.

The discovery of a monocytosis is a frequent phenomenon, requiring confirmation by reading under a microscope by an experimented biologist, to overcome usual cytological traps such as the presence of hairy cells, promonocytes or monoblasts. In the vast majority of cases the secondary origin is very easily found by the context and/or the presence of a biological inflammatory syndrome. More rarely the diagnosis is directed towards an eosinophilic pathology or an acute leukemia. In other cases, CMML, MPN or MDS with monocytosis may be highlighted. In the absence of any pathognomonic element and the presence of "borderline" forms the differential diagnosis between these 3 entities is not always straightforward, requiring, according to WHO, molecular investigations and elimination of any reactive cause of monocytosis. Although histological, immunohistochemical and phenotypic flow cytometric studies are not currently recommended by WHO, these investigations could be of interest in the evaluation of difficult cases.

The presence of serum monoclonal IgM is often associated with the diagnosis of Waldenström macroglobulinemia (WM) or other chronic lymphoproliferative disorders. IgM myeloma is a rare entity (0.5%). We report the case of an IgM myeloma complicated by systemic amyloidosis AL, with an impure nephrotic syndrome and a factor FX deficiency.

The most frequent causes of hemolytic anemias are immune or infectious diseases, drug induced hemolysis, thrombotic microangiopathies, hereditary spherocytosis, glucose-6-phosphate dehydrogenase or pyruvate kinase deficiencies, thalassemia's and sickle cell disease. Sometimes no cause is found because a rarer etiology is involved. The goal of this review is to remember some unfrequent constitutional or acquired causes and to point out difficulties to avoid wrong interpretations of analysis results.