Evans syndrome as initial presentation of COVID-19 infection: A case report and review of the literature / Le syndrome d'Evans comme présentation initiale du Covid-19 : à propos d'un cas et revue de la littérature

Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by the concomitant or sequential association of auto-immune hemolytic anemia (AIHA) with immune thrombocytopenia (ITP), and less frequently autoimmune neutropenia. Coronavirus disease 2019 (Covid-19) may cause various hematologic conditions. COVID-19 may also induce Evans syndrome via immune mechanisms. Here, we describe the case of a patient developing Evans syndrome as initial presentation of Covid-19 infection.

Hematological hypereosinophilia / Hyperéosinophilies hématologiques

Introduction: Hematological disorders are the third cause of hypereosinophilia, after allergic and parasitic disease. The objective of our study is to show the epidemiological, clinical, biological and therapeutic characteristics of hematological hypereosinophilia. Patients and methods: This is a retrospective study over a 4-year period (March 2017-March 2021) concerning 14 patients with hematological hypereosinophilia. Results: Fourteen patients were included (9 women and 5 men). The median age at diagnosis was 55 years. Hematological hypereosinophilia was mainly of clonal etiology. Clinical manifestations were either specific to hypereosinophilia, such as organ damage, or related to the etiology of hypereosinophilia, such as hepato-splenomegaly and lymphadenopathy. Moderate and severe forms were the most commonly reported. Several other quantitative and qualitative abnormalities of the blood picture were reported. Identification of the FIP1L1-PDGFRA fusion gene by molecular biology (RT-PCR) was positive in two patients. Imatinib was the main treatment for clonal forms. Conclusion: Hematological hypereosinophilia is a rare and complex pathology. Our study has confirmed this epidemiological, clinical, biological and therapeutic heterogeneity and has enabled us to realize the contribution of molecular biology in the diagnosis and the treatment of hypereosinophilia.

Introduction: Les affections hématologiques représentent la troisième cause des hyper éosinophilies. L'objectif de notre travail est d'étudier les caractéristiques épidémiologiques, étiologiques, cliniques, biologiques et thérapeutiques des hyperéosinophilies hématologiques. Patients et méthodes: Il s'agit d'une étude rétrospective menée sur une période de 4 années (mars 2017-mars 2021). Cette étude a colligé 14 patients atteints d'hyperéosinophilie hématologique. Résultats: Quatorze patients ont été inclus (9 femmes et 5 hommes). L'âge médian au moment du diagnostic était de 55 ans. L'hyperéosinophilie hématologique était principalement d'étiologie clonale. Les manifestations cliniques étaient soit spécifiques à l'hyperéosinophilie telles que les atteintes d'organes, soit liées à l'étiologie de l'hyperéosinophilie telles que l'hépato-splénomégalie et les adénopathies. Les formes modérées et sévères étaient les plus fréquemment rapportées. Plusieurs autres anomalies quantitatives et qualitatives de l'hémogramme ont été rapportées. L'identification du gène de fusion FIP1L1-PDGFRA par biologie moléculaire (RT-PCR) était positive chez deux patients. L'imatinib était le traitement de choix des formes clonales. Conclusion: L'hyperéosinophilie hématologique est une pathologie rare et complexe. Notre étude a permis de confirmer cette hétérogénéité épidémiologique, étiologique, clinique, biologique et thérapeutique et de montrer l'apport de la biologie moléculaire dans le diagnostic et le traitement de cette pathologie.

Acquired von Willebrand syndrome: Five cases report and literature review.

Acquired von Willebrand syndrome is a rare bleeding disorder with laboratory findings similar to those of inherited von Willebrand disease. Principal factors distinguishing acquired von Willebrand syndrome from the latter condition include lack of prior bleeding disorders, diagnosis at older age, negative family history and association with underlying conditions. METHODS: Retrospective, monocentric descriptive case series of acquired von Willebrand syndrome diagnosed between 2010 and 2020. Diagnostic criteria included a recent history of bleeding, a negative family history and a presence of underlying disorders. RESULTS: Five men were diagnosed with acquired von Willebrand syndrome. All of them presented with recent mucocutaneous bleeding. The biological phenotype was a type 2 von Willebrand disease in all cases, with decreased VWF:RCo/VWF:Ag and VWF:CB/VWF:Ag ratios (<0.7). Lymphoproliferative, autoimmune and cardiovascular diseases were the main underlying conditions identified. Screening for an anti-von Willebrand factor inhibitor was positive in two patients. Four patients received treatment for the underlying disorder. High-dose intravenous immunoglobulins were the most frequent treatment administrated. Improvement of plasma von Willebrand factor levels was observed in four cases. CONCLUSION: Acquired von Willebrand syndrome is a rare but potentially serious disease. The diagnostic should be suspected in adults with unusual mucocutaneous bleeding associated with lymphoproliferative, myeloproliferative, autoimmune and cardiovascular disorders.

Syndrome de Willebrand acquis : une pathologie rare au diagnostic difficile.

Le syndrome de Willebrand acquis (SWa) est un syndrome hémorragique rare dont la fréquence est probablement sous-estimée. Les signes cliniques et le bilan d'hémostase sont comparables à ceux de la maladie de Willebrand héréditaire, mais les patients atteints du SWa n'ont pas d'antécédents hémorragiques, personnels ou familiaux. Il s'agit le plus souvent de personnes âgées et ce sont les saignements cutanéomuqueux qui sont les plus fréquents. Parmi les affections sous-jacentes à l'origine de ce syndrome, les plus fréquentes sont les gammapathies monoclonales, les désordres lymphoprolifératifs, les maladies auto-immunes et les pathologies cardiovasculaires. L'apparition du SWa est liée soit à la présence d'auto-anticorps dirigés contre le facteur von Willebrand (vWF), neutralisant son activité ou accélérant sa clairance, soit à une protéolyse augmentée, soit à une destruction mécanique en présence de forces de cisaillement élevées, soit à une adsorption sur des cellules tumorales ou des plaquettes activées. Le diagnostic du SWa est compliqué du fait de la nécessité d'associer de multiples tests biologiques. La prise en charge thérapeutique est basée sur le traitement de la pathologie sous-jacente et la prévention des saignements.

[Cytological and cytogenetic profile of 378 cases of acute myeloid leukemia in a Tunisian center].

INTRODUCTION: Acute leukemia (AL) represents the first hematological malignancy diagnosed and treated in Tunisia. OBJECTIVE: To describe the demographic, cytological, cytogenetic and prognostic characteristics of acute myeloid leukemia (AML) in the Tunisian center over a period of 11 years. METHODS: A retrospective study was performed on a series of AML cases diagnosed at Farhat Hached Hospital in Sousse, between January 2009 and December 2019. Cytological analysis according to the French-American-British classification and cytogenetic and molecular analysis allowed to classify AML according to the World Health Organization recommendations of 2016. The prognosis was established according to the recommendations of European Leukemia Net. RESULTS: The diagnosis of AML was confirmed in 378 cases with a median age at diagnosis of 43 years and a sex ratio of 1.32. AML with maturation was observed in 31% of cases. Recurrent abnormalities were detected in 25% of karyotypes, dominated by the t(15;17) translocation. The latter was associated with 75% of promyelocytic LA. Cytogenetic abnormalities associated with myelodysplasias were detected in 17% of cases, 59% of which had a complex karyotype. AMLs without specificity accounted for 57% of AMLs. Furthermore, 55% of patients had an intermediate prognosis. CONCLUSION: The lack of a Tunisian registry of hematological malignancies and the increasing incidence of AML, require epidemiological studies to establish the cytological and cytogenetic profile of the tunisian population. This will allow us to reinforce the diagnostic and therapeutic means with the ultimate goal of improving the survival of patients.

Hemophagocytic lymphohistiocytosis: an unusual complication of leprosy.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition of chaotic uncontrolled immune system stimulation and not fully understood pathophysiology. Most reported cases of hemophagocytic syndrome in patients with mycobacterial infections have been associated with Mycobacterium tuberculosis. As far as we could ascertain, to date, no established HLH case complicating leprosy has been published in the medical literature. CASE REPORT: We describe here a new case of Hansen's disease in a 58-year-old Tunisian man with an unusual complicated clinical course documented as hemophagocytic syndrome. Cutaneous and neurological involvements were the main clinical signs of Hansen's disease. Histological findings suggested the diagnosis of leprosy and were somewhat more characteristic of the lepromatous leprosy type. While on antileprosy treatment, he developed unexplained persistent fever, organomegaly, bicytopenia, and elevated rate of inflammatory markers with bone marrow aspirate showing large macrophages with increased phagocytosis of mature and immature blood elements, typical features of hemophagocytic syndrome. CONCLUSION: A high index of suspicion is essential for prompt diagnosis of hemophagocytic syndrome in the setting of disseminated infection such as leprosy.

[Platelet satellitism during cutaneous leishmaniasis]. / Satellitisme plaquettaire au cours d'une leishmaniose cutanée.

Platelet satellitism is considered an uncommon phenomenon with an estimated frequency at 0.008%. About 100 cases have been published. If not recognized, this artifact can lead to an erroneous diagnosis of thrombocytopenia. We report the case of a patient, with cutaneous leishmaniasis, who developed an isolated thrombocytopenia. The blood smear prepared from peripheral blood sample collected with ethylenediaminetetraacetic acid showed platelets rosetting around polymorphonuclear. This phenomenon disappeared after treatment with Glucantime® for fifteen days and improvement of the lesions. We discuss also possible mechanisms to better understand this phenomenon.

Chronic myeloid leukemia as a secondary malignancy after lymphoma in a child. A case report and review of the literature.

BACKGROUND: Philadelphia chromosome-positive chronic myeloid leukemia (CML) in children is very rare. CML occurring as a secondary malignancy in individuals treated for diffuse large B-cell lymphoma (DLBCL) is also rare. CASE REPORT: We present the case of a 5-year-old female patient who developed a right orbital mass that was diagnosed as DLBCL. 9 months after receiving treatment for DLBCL, she presented with a white cell count of 250,000/mm(3). Peripheral blood and bone marrow (BM) evaluation revealed a myeloproliferative disorder. Cytogenetic and molecular studies demonstrated the presence of t(9;22). CML following DLBCL has not been previously described in the younger population. To our knowledge, this is the first report of a child who developed a CML as a second malignancy after DLBCL. Therapy-related CML and non-therapy-related secondary CML are discussed as potential explanations of this highly unusual clinical presentation. CONCLUSION: Hematological disorders such as CML may occur after lymphomas. With the increased use of BM cytogenetic studies during staging for lymphoid malignancies, future studies may be able to clarify the question of whether the CML clone in some of these patients existed before treatment for lymphoma.