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Introduction: Monoclonal gammopathies are a group of disorders associated with monoclonal proliferation of plasma cells that produces a monoclonal protein. To describe the epidemiological and immunochemical characteristics of monoclonal gammopathies diagnosed during a nineteen-year period in a Moroccan teaching hospital was the main objective of this study. Methods: This study was performed from January 2000 to August 2019. It was a retrospective study that included of 545 Moroccan patients with monoclonal gammopathy. Results: The patients who participated in the study, 374 (68.6%) were male and 171(31.4%) were female, with a mean ±SD age of 62.24±13.14 years. The most frequent reasons for admission were bone pain (41,60%), renal failure (19.08%), alteration of the general condition (12.21%) and anemia (10.69). Plasma cell proliferative disorders in our study were as follow, multiple myeloma (MM) (45.65%), Monoclonal gammopathies of undetermined significance (MGUS) (39.05%), Waldenstrom’s macroglobulinemia(5.58%), Lymphoma (2.27%+1.2%), Chronic Lymphocytic Leukemia (2.48%), Plasma cell leukemia (1.86%), Plasmacytoma (0.62%), POEMS syndrome (0.41%), and Amyloidosis (0.84%). The most frequent isotypes in MM were the IgGκ (62) 36.5%, IgGλ(52)30.6%, IgAκ(27)15.9% and the IgAλ (19)11.2%. It is also worthy of note, that Free light chain MM represents 20% of all cases of MM. Conclusions: This is the largest Moroccan cohort, it included 545 patients. The results of this study point to the need for an early diagnosis of monoclonal gammopathies in the Moroccan population

Cold agglutinin are erythrocyte antibodies which possess the property of agglutinating red blood cells at temperatures of below 37°C, this phenomenon is reversible after heating. This is usually immunoglobulin M (IgM) class. Their pathogenicity is much more related to their temperature range of activity than their title. As we report in this observation, cold hemagglutination makes it difficult to interpret certain immunological tests such as ABO Rh blood grouping or searching for irregular antibodies (SAI). The discovery of cold agglutinins can be fortuitous revealing itself by disturbances and aberrations in the results of blood count or as part of a suggestive clinical or laboratory table cold hemagglutinin disease. The search for a lymphoid hematological at their diagnosis should be systematic.

L´érythrophagocytose blastique correspond à une hyperactivation des blastes. L´érythrophagocytose est retrouvée dans les hémopathies myéloïdes surtout avec la t (8;16). Dans ce travail nous présentons un cas exceptionnel d´érythrophagocytose blastique au cours d´une leucémie aigue lymphoblastique T sans anomalies cytogénétiques. A.Z âgée de 19 ans, l´examen à l´admission a trouvé un syndrome fébrile avec des vertiges et phosphènes, un syndrome tumoral avec une hypertrophie amygdalienne et gingivale. L´hémogramme a objectivé une hyperleucocytose (399,5 G/L), avec une anémie arégénérative (Hb: 9,3 g/dl) et thrombopénie (plaquettes: 40 G/L). Le myélogramme a montré 90% des blastes (MPO négative) avec des images d´érythrophagocytose blastique. L´immunophénotypage a confirmé une LAL T. L´analyse cytogénétique était normale. L´érythrophagocytose blastique dans une LAL T semblerait être une entité distincte nécessitant la précision de l´impact de ces images sur le diagnostic, le pronostic voir même le traitement des LAL T.

Nous rapportons un cas unique d´une polyradiculonévrite inflammatoire démyélinisante chronique paranéoplasique secondaire à un lymphome non hodgkinien T de typenatural killernasal.

The initiation of treatment with acenocoumarol is a critical phase that can lead to an iatrogenic event in some patients carrying polymorphisms of the genes involved in the response to treatment, notably VKORC1 and CYP2C9. We report the first case in Morocco and Africa of hypersensitivity to acenocoumarol at the initiation dose in a 70-year-old patient who required an extremely low maintenance dose (0.25 mg / day) to achieve INR target. Genotyping results showed that the patient was homozygous mutated for (VKORC1) -1639 G> A and (VKORC1) -11173 C> T and heterozygous for (CYP2C9) * 2 and (CYP2C9) * 3 demonstrating extreme sensitivity to acenocoumarol due to the cumulative effect of these genetic polymorphisms on the maintenance dose of the anticoagulant. Our study shows the major benefit of prospective genotyping of CYP2C9 and VKORC1 prior to initiation of acenocoumarol treatment, as a good predictor of extreme susceptibility to VKA.

Increased prevalence of latent tuberculosis infection (LTBI) has been observed among high-risk populations such as healthcare workers (HCWs). The results may depend on the method of LTBI assessment, interferon-gamma release assay (IGRA) and/or tuberculin skin test (TST). Here, we investigated the prevalence and risk factors for LTBI assessed by both IGRAs and TST in HCWs living in Morocco, a country with intermediate tuberculosis (TB) endemicity and high BCG vaccination coverage. HCWs were recruited in two Moroccan hospitals, Rabat and Meknes. All the participants underwent testing for LTBI by both IGRA (QuantiFERON-TB Gold In-Tube, QFT-GIT) and TST. Different combinations of IGRA and TST results defined the LTBI status. Risk factors associated with LTBI were investigated using a mixed-effect logistic regression model. The prevalence of LTBI among 631 HCWs (age range 18-60 years) varied from 40.7% (95%CI 36.9-44.5%) with QFT-GIT to 52% (95%CI 48.2-56.0%) with TST using a 10 mm cut-off. The highest agreement between QFT-GIT and TST (κ = 0.50; 95%CI 0.43-0.56) was observed with the 10 mm cut-off for a positive TST. For a definition of LTBI status using a double positive result for both QFT-GIT and TST, significant associations were found with the following risk factors: being male (OR = 2.21; 95%CI 1.40-3.49; p = 0.0007), belonging to age groups 35-44 years (OR = 2.43; 95%CI 1.45-4.06; p = 0.0007) and even more 45-60 years (OR = 4.81; 95%CI 2.72-8.52; p = 7.10-8), having a family history of TB (OR = 6.62; 95%CI 2.59-16.94; p = 8.10-5), and working at a pulmonology unit (OR = 3.64; 95%CI 1.44-9.23; p = 0.006). Smoking was associated with LTBI status when defined by a positive QFT-GIT result (OR = 1.89; 95%CI 1.12-3.21; p = 0.02). A high prevalence of LTBI was observed among HCWs in two Moroccan hospitals. Male gender, increased age, family history of TB, and working at a pulmonology unit were consistent risk factors associated with LTBI.

Les lymphomes non hodgkiniens représentent un groupe hétérogène caractérisé par la prolifération maligne des cellules lymphoïdes. Cette affection particulièrement fréquente entre 18 et 40 ans peut être rencontrée lors d’une grossesse, représentant ainsi l’un des principaux problèmes diagnostiques et thérapeutiques rencontrés par l’obstétricien et l’hématologue. Nous rapportons deux cas de lymphome non hodgkinien diagnostiqués et traités au cours de la grossesse ; ainsi qu’une revue de la littérature discutant les modalités de prise en charge possibles au cours de ces grossesses considérées à haut risque.

We report a case of dramatic outcome of severe haemolytic disease in a newborn due to RH1 incompatibility. A newborn with A RH1 blood group was admitted in the Mohammed V Military Teaching Hospital for the problem of hydrops fetalis associated with RH1 incompatibility. The blood group of his mother, aged 31, was AB RH1-negative and that of his 37 year old father was A RH1.The mother had a history of 4 term deliveries, 3 abortions, and 1 living child. There was no prevention by anti-D immunoglobulin postpartum. The mother‘s irregular agglutinin test was positive and the pregnancy was poorly monitored. The laboratory tests of the newborn showed a high total serum bilirubin level (30 mg/L) and macrocytic regenerative anemia (Hemoglobin=4 g/dL, mean corpuscular volume = 183 fL, reticulocytes count =176600/m3). The blood smear showed 1256 erythroblasts per 100 leukocytes, Howell–Jolly bodies and many macrocytes. The direct antiglobulin test was positive. He was transfused with red blood cell concentrates and treated with conventional phototherapy. The evolution was unfavourable; he died three days after the death of his mother. The monitoring of these high-risk pregnancies requires specialized centers and a close collaboration between the gynaecologist and the blood transfusion specialist to strengthen the prevention, as well as clinico-biological monitoring in patients with a history of RH1 fetomaternal alloimunization.

Introduction: Non-Hodgkin's lymphoblastic B lymphoma with lymph node location is exceptional in children. Unlike acute lymphoblastic leukemia, which is the first cancer in children around the age of 3 with a favorable prognosis with a survival of 5 years in 90%. Observation: child, 15 years old. The hospital admission examination showed a febrile patient (40°C), bilateral cervical and inguinal polyadenopathies, the absence of hepatosplenomegaly. The hemogram at entry found bicytopenia made up of angerogenic anemia and thrombocytopenia. On the haematological level, there has been an evolution towards pancytopenia. A lymph node biopsy showed medullary infiltration with lymphoma cells of phenotype B. The karyotype found hyper diploidy with t (9.22). The patient was put on corticosteroid therapy, hyperhydration and (Allopurinol) followed by COP type chemotherapy (Cyclophosphamide, Oncovin, Prednisone) with good tolerance. A year later the patient presented with a feverish peak (40°C). Only a hematological relapse in the form of acute leukemia. Therapeutically, it was decided to re-induce the patient according to the GRAALL catch-up protocol (Idarubicin-Aracytine) and to propose it for an allograft of bone marrow, but unfortunately the patient died. Conclusion: Lymphoblastic lymphoma / Acute lymphoblastic leukemia B, associated with t (9; 22) and hyperdiploidy in children is an exceptional hemopathy and has a negative diagnosis.

Background The acquired inhibitors of coagulation have been observed in very rare cases of monoclonal gammopathies. We report a very rare case of anti-factor XI antibodies in patient with plasma cell leukemia (PCL). Case presentation This is a 59-year-old male patient without pathological history, admitted to the nephrology department for management of renal insufficiency and anemia syndrome. The history and physical examination revealed stigmata of hemorrhagic syndrome including hemothorax and hemoptysis. The hemostasis assessment showed an isolated prolonged activated partial thromboplastin time (APTT) with APTT ratio = 2.0.The index of circulating anticoagulant (37.2%) revealed the presence of circulating anticoagulants. The normalized dilute Russell viper venom time ratio of 0.99 has highlighted the absence of lupus anticoagulants. The coagulation factors assay objectified the decrease of the factor XI activity corrected by the addition of the control plasma confirming the presence of anti-factor XI autoantibodies. In addition, the blood count showed bicytopenia with non-regenerative normocytic normochromic anemia and thrombocytopenia. The blood smear demonstrated a plasma cell count of 49% (2842/mm3) evoking PCL. The bone marrow was invaded up to 90% by dystrophic plasma cells. The biochemical assessment suggested downstream renal and electrolyte disturbances from exuberant light chain production with abnormalities including hyperuricemia, hypercalcemia, elevated lactate dehydrogenase, non nephrotic-range proteinuria and high level of C reactive protein. The serum protein electrophoresis showed the presence of a monoclonal peak. The serum immunofixation test detects the presence of monoclonal free lambda light chains. He was treated with velcade, thalidomide and dexamethasone. The patient died after 2 weeks despite treatment. Conclusion Both PCL and anti-factor XI inhibitors are two very rare entities. To the best of our knowledge, this is the first reported case of a factor XI inhibitor arising in the setting of PCL. Factor inhibitors should be suspected in patients whose monoclonal gammopathies are accompanied by bleeding manifestations.

Hemoglobin C is a hemoglobin variant encountered worldwide. The regionswith high prevalence are West Africa and South-East Asia.The objective of this study is to report cases of hemoglobin C disease brought together during these last twelve years in the Laboratory of Biochemistry and Toxicology of RabatMilitary Hospital Mohammed V (MHIMV).- This was a retrospective study including111 cases of hemoglobin C disease collected in the Laboratory of Biochemistry of the MHIMVover the past 12 years. A questionnairewasfulfilledwith the epidemiological data,clinical data and the results of the biological explorations. The screening of the hemoglobin variant in this study included several biochemical (hemoglobin electrophoresis at acid and alkalinepH) and hematological tests.- Sex-ratio was equal to 1,22. The age at the time of diagnosis ranges between 4 and 80years old, with the mean of 38. North-West regions of Morocco seem most affected. The most frequent reasons for prescription of the hemoglobin's studywere: biological abnormalities, splenomegaly and anemic syndrome. Blood smear reveals frequently anisopoikilocytosis and red blood target. The biochemical tests contribute to the diagnosis and reveal various and varied etiological groups: heterozygous A/C (75%),homozygous C/C (8%), double heterozygous S/C (9%),C/β+-thal (6%) andC/O-Arab (2%). Conclusion - The results of the present descriptive study are in line with the literature data. The importance of genetic counseling and the installation of a national card of systematic neonatal tracking seemto be unavoidable.

We investigated the impact of non-genetics factors, and single nucleotide polymorphisms (SNPs) in VKORC1, CYP2C9, CYP4F2, and GGCX on acenocoumarol dosage in Moroccan adult's patients, in order to develop an algorithm to predict acenocoumarol dose for Moroccan patients. Our study consisted of 217 Moroccan patients taking a maintenance dose of acenocoumarol for various indications. The patients were genotyped for VKORC1 -1639 GA, VKORC1 1173 CT, CYP2C9*2, CYP2C9*3, CYP4F2 1347 GA and GGCX 12970 CG SNPs. The statistical analysis was performed using the SPSS software. The age and SNPs in VKORC1 and CYP2C9 were significantly associated with the weekly acenocoumarol dose requirement (p = 0.023, p = 0.0001 and p = 0.001 respectively). There was no association found between the weekly acenocoumarol dose and the CYP4F2 or GGCX variants (p-value0.05). Non-parametric analysis confirmed the accumulate effect of variant alleles at VKORC1 -1639 GA, VKORC1 1173 CT and CYP2C9 SNPs on the acenocoumarol dose requirement. With 90.24% less dose required for one patient carrying homozygote variant at VKORC1 -1173 (TT) and CYP2C9 *x/*x haplotype. The multiple linear regression analysis showed that mutation in VKORC1 -1639, VKORC1 1173 SNPs, or in CYP2C9 haplotype reduces the mean acenocoumarol weekly dose to 25.4%, 23.4% and 6.2%, respectively. The R2 for multiple regression analysis final model was found to be 35.9%. In this work we were able to establish the factors influencing interindividual sensitivity to the anticoagulant therapy that can help physicians to predict optimal dose requirement for long term therapy.

Prognostic scores are an indispensable tool in the management of myelodysplastic syndromes.Identify prognostic factors influencing overall survival of patients with myelodysplastic syndromes.We included all patients with myelodysplastic syndromes treated in the clinical hematology department of the military hospital of Rabat (Morocco).Seventy-four patients were included. The sex ratio [M / F] was 1.8. The median age was 65.5 years. The median follow-up was 45 months. The karyotype prognostic was good in 74%. In univariate analysis, prognostic factors influencing overall survival are: Age (p = 0.024), karyotype (p = 0.033), rate of bone marrow blasts (p = 0.002), transfusion dependence (p = 0.002), number of cytopenias (p = 0.001), hemoglobin (p = 0.009) and neutrophil count (p = 0.003). In multivariate analysis the only prognostic factor that influenced overall survival was the karyotype (p = 0.036). Transfusion dependence (p = 0.072) and the rate of marrow blasts (p = 0.107) was close to significance. The WHO Prognostic Scoring System (WPSS), the International Prognostic Scoring System (IPSS) and the Revised International Prognostic Scoring System (RIPSS) influence overall survival with a statistical significance (p0.001).Our study confirms the prognostic value of the karyotype and validated the WPSS, the IPSS and the RIPSS scores as significant predictors of overall survival in Moroccan patients with myelodysplastic syndromes.

Background The retinopathy is an uncommon complication in individuals with sickle cell trait except for the cases of sickle cell trait associated with systemic arterial hypertension, diabetes mellitus, syphilis, tuberculosis and sarcoidosis. Case Presentation A retinopathy in a 16 year-old child with no history of consanguinity in the parents revealed a sickle S trait associated to heterozygous alpha thalassemia. His mother has Sickle cell anaemia (Hb SS) and his father is a carrier of heterozygous alpha-thalassemia status that it was unknown before. Conclusion This case report describes a proliferative retinopathy in a 16 year-old patient with co-inheritance of heterozygous alpha + −thalassemia and sickle trait.

Background Survival is increasing after early breast cancer revealing frequent relapses and possibility of developing secondary malignancies. The concomitant occurrence of these two events is exceptionally disastrous and lethal. We report a case of a Moroccan woman who was successfully managed for synchronous recurrent breast carcinoma and chronic myelogenous leukemia. Case presentation A 42-year-old Moroccan woman was diagnosed with localized breast carcinoma in 2008. She received six cycles of an adjuvant chemotherapy regimen, radiation therapy and hormonal therapy by tamoxifen. After completion of 5 years of tamoxifen our patient reported asthenia; a physical examination found hepatomegaly, massive splenomegaly measuring 21 cm and supraclavicular lymphadenopathy. The staging showed lung and liver metastases. Morphology and immunohistochemical profile of this metastasis identified an adenocarcinoma of mammary origin. In parallel, the diagnosis of chronic myeloid leukemia was suspected because of the presence of a leukocytosis at 355 × 109/L, with circulating blasts of 4%. Chronic myeloid leukemia was confirmed by a bone marrow biopsy with the presence of Ph chromosome on cytogenetical analysis. Daily imatinib was ordered concurrently with chemotherapy-type docetaxel. The metastases were stable after nine courses of chemotherapy. Due to breast cancer progression 4 months later, bevacizumab and capecitabine were introduced. A major molecular response was achieved after 12 and 18 months. She has now completed 2 years of follow-up, still on a major molecular response, and is undergoing imatinib and capecitabine treatment. Conclusions Leukocytosis in breast cancer patients can reveal chronic myeloid leukemia. It may warrant a workup to find the underlying etiology, which could include a secondary hematological malignancy.

Griscelli syndrome type 2 is a rare autosomal recessive disorder, due to a mutation in RAB27A gene. It associates partial albinism, silver hair and immune deficiency. We report the case of a 6 year-old boy who was admitted to the Emergency department with severe sepsis complicated by hemophagocytic syndrome. Many clinical and biological criteria leads to the diagnosis of type 2 Griscelli syndrome: consanguineous family, recurrent infection, absence of psychomotor retardation, oculocutaneous albinism, silver hair, occurrence of hemophagocytic syndrome and especially the pathognomonic appearance on microscopic examination of the hair. The absence of giant organelles inclusion in all granulated cells eliminated Chediak-Higashi syndrome.

Background Hemophagocytic lymphohistiocytosis in adults is often secundary to an infection or a neoplasm. In this last case, T cell lymphomas are the most frequent causes. Hemophagocytic lymphohistiocytosis secundary to a B cell lymphoma has been rarely reported. Case presentation We describe a case of a hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma treated with conventionnal chemotherapy leading to a complete remission. Conclusion Prompt etiologic diagnosis and treatment of hemophagocytic lymphohistiocytosis leads to satisfactory outcome.

La leucémie à plasmocytes est une hémopathie maligne rare définie par la présence de plus de 20 % de plasmocytes de la formule leucocytaire ou un nombre de plasmocytes circulants supérieur à 2 × 109/L (2G/L). Elle peut être primitive, dans 60% des cas, et se manifeste d'emblée sur un mode leucémique ou secondaire, dans 40% des cas, compliquant un myélome multiple connu. Vu la rareté de cette affection, seuls quelques cas ont été rapportés dans la littérature. Elle est caractérisée par son agressivité et son mauvais pronostic. A travers 03 cas diagnostiqués au laboratoire d'hématologie de l'hôpital militaire Mohammed V les auteurs présentent les particularités cliniques, biologiques et pronostic de cette affection.The Pan African Medical Journal 2016;24

Les brules graves presentent souvent des anemies plus au moins profondes, necessitant des transfusions dans les premiers jours qui suivent l'accident. C'est une anemie hemolytique d'origine extra-corpusculaire non auto-immune. Sur le frottis sanguin, des anomalies morphologiques des globules rouges ont ete decrites suite au choc thermique, des spherocytes, des schizocytes et des echinocytes. A travers ce cas, nous dressons une revue de la litterature des mecanismes probables des anemies hemolytiques dans les services des brules.

Resume La leucemie a plasmocytes est definie par la presence de plus de 20 % de plasmocytes dans les frottis de sang peripherique ou un nombre de plasmocytes circulants superieur a 2 G/L. C’est une forme particuliere et agressive du myelome multiple qui possede deux variantes : - la forme secondaire : compliquant une maladie de Kahler connue ; - la forme primitive : observee de novo pouvant reveler un myelome jusque la meconnu. Cette derniere a des points communs avec le myelome multiple et possede certaines particularites. A la lumiere de deux observations diagnostiquees au service d’hematologie de l’Hopital Militaire d’Instruction Mohammed V de Rabat, les auteurs comparent les formes primitives des leucemies a plasmocytes aux formes secondaires et au myelome multiple.

Plasma cell leukemia (LP) is a rare hematologic malignancy. Its prognosis is very derogatory. It is defined by the presence in circulating blood of more than 2 G/L plasmocytes or greater than 20% of the total leukocytes. It comes in two forms: secondary plasma cell leukemia complicating multiple myeloma (MM) and primary setting. Its incidence is estimated at 0.9% of patients with acute leukemia and 2-4% of patients with MM. We report, through three observations, the clinical presentation of the plasma cell leukemia, its cytological features, immunophenotypic, physiopathological and therapeutic care.

Introduction. We report the case of a Bing and Neel syndrome revealed by an isolated left ptosis.Case Report. a 57-year-old man was followed up since October 2003 for a typical Waldenström’s macroglobulinemia. A first complete remission was obtained with chlorambucil. In August 2004, he relapsed. A second complete remission was obtained with RFC chemotherapy regimen (rituximab, fludarabine, and cyclophosphamide). In October 2009, the patient presented with an isolated left ptosis revealing a Bing and Neel syndrome. The diagnosis was suspected on MRI and confirmed by the detection in the CSF of a monoclonal IgM similar to the one found in the plasma. A quite good partial remission has been obtained after one course of RDHAP (rituximab, dexamethasone, cytarabine, and cisplatin) and 3 courses of RDHOx (rituximab, dexamethasone, cytarabine, and oxaliplatin), in addition to ten intrahectal chemotherapy injections. The treatment was followed by intensification and autologous stem cell transplantation. At D58, the patient died due to a septic shock.Conclusion. BNS is a rare and potentially treatable complication of WM. It should be considered in patients with neurologic symptoms and a history of WM.

The thrombotic microangiopathy is a syndrome characterized by the combination of mechanical hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. In addition to the idiopathic form, several cases are identified as secondary to pregnancy, infections, disease systems, organ transplants, and cancer. Other forms are secondary to drugs including antimitotics. We report the case of a patient followed for acute myelogenous leukemia. She received induction chemotherapy combining daunorubicin and cytarabine, complicated by thrombotic thrombocytopenic purpura.

Mechanisms of non-immune haemolytic anemia in burn patients: severely burned patients often present with anemia in the early course of resuscitation, requiring the transfusion of red blood cells. One of the causes is a non-immune haemolytic anemia. On the blood smear, there are morphological abnormalities of the red blood cells such as the presence of spherocytes and schizocytes. This case report is an opportunity to review the existing literature on the possible mechanisms of haemolytic anemia during severe burns.