A retinopathy in young patient with co-inheritance of heterozygous alpha + -thalassemia and sickle trait: a case report.

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.

Hemoglobinosis C in Morocco : A report of 111 cas.

BACKGROUND: - 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). METHODS: - 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. RESULTS: - 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/ß+-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.

Prognostic factors predicting overall survival in Moroccan patients with myelodysplastic syndrome.

BACKGROUND: Prognostic scores are an indispensable tool in the management of myelodysplastic syndromes. AIM: Identify prognostic factors influencing overall survival of patients with myelodysplastic syndromes. METHODS: We included all patients with myelodysplastic syndromes treated in the clinical hematology department of the military hospital of Rabat (Morocco). RESULTS: 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 (p <0.001). CONCLUSION: 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.

Epidemiology of monoclonal gammopathy in Morocco - A hospital-based study.

BACKGROUND: Monoclonal gammopathies are a group of disorders associated with clonal proliferation of plasma cells that produces a monoclonal protein. AIMS: The main objective of this study was to describe the epidemiological and immunochemical characteristics of monoclonal gammopathies diagnosed during 19 years in a Moroccan teaching hospital. MATERIALS AND RESULTS: This retrospective study enrolled 443 Moroccan patients with monoclonal gammopathy, patients meeting the inclusion and exclusion criteria in at the biochemistry department of Military Hospital in Rabat, the capital of Morocco, from January 2000 to August 2019. Of the 443 enrolled patients, 320 (72.23%) were men and 123 (27.77%) were women. All patients were of Caucasian origin, from 12 Moroccan regions. The patient's samples were collected and subjected to serum protein electrophoresis and serum immunofixation electrophoresis to further characterize the monoclonal protein. The mean ± SD age of the 443 participants was 62.24 ± 13.14 years. Reasons for being admitted to the hospital were as follows, 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 follows, 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 worth noting that Free light chain MM represents 20% of all cases of MM. CONCLUSIONS: We found that monoclonal gammopathies are age-related and affects men more than women, also the results of this study point to the delayed diagnosis of monoclonal gammopathies, since most of our patients were diagnosed at the MM stage. The most frequent isotypes were the IgGκ and IgGλ in MM and MGUS, in Waldenström macroglobulinemia were IgMκ and IgMλ and the oligoclonal profile represented only 3.70%.

Cold agglutinins revealed by abnormalities to the cell blood count: a case report.

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.

[The mechanism of non-immune haemolytic anaemia in burns patient]. / Mécanismes de l'anémie hémolytique aiguë non immunologique chez le brûlé grave.

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.

[IAssociation between Kaposi´s sarcoma and multiple myeloma: is it caused by HHV-8?] / Sarcome de kaposi et myélome multiple: s´agit-il d´une association causée par le HHV-8?

Kaposi´s sarcoma is a tumor characterized by purple or brownish lesions affecting the skin. It is most commonly associated with human herpes virus type 8 (HHV-8) infection and may be secondary to malignant hemopathy, including lymphomas. We here report a new case of a very rare combination: Kaposi´s disease-multiple myeloma. The study involved Mr. aged 67 years, treated for Kaposi´s disease in the Department of Dermatology. Serological test for HHV-8 was positive; it was associated with stage I multiple myeloma IgG Lambda with a poor prognosis. We here report this 21st case of Kaposi-Kahler in order to highlight Kaposi-human herpes 8 virus variant involved in the pathophysiology of multiple myeloma. More studies are needed in order to establish this exceptional link.

[Erythrophagocytosis by blast cells and de novo T cell LAL without cytogenetic abnormalities in a Moroccan patient]. / Un cas marocain d'érythrophagocytose blastique et LAL T de novo sans anomalie cytogénétique.

Erythrophagocytosis by blast cells is due to hyperactivation of blast cells. Erythrophagocytosis is associated with T cell myeloid hemopathies (8;16). This study shows an exceptional case of erythrophagocytosis by blast cells in a patient with acute T-lymphoblastic leukemia without cytogenetic abnormalities. We here report the case of A.Z, aged 19 years presenting with febrile syndrome with dizziness and phosphenes, tumor syndrome with amygdala and gingival hypertrophy. Blood count revealed hyperleukocytosis (399.5 G/L), with aregenerative anemia (Hb: 9.3 g/dl) and thrombocytopenia (platelet count: 40 g/L). Myelogram showed 90% of blast cells (MPO-negative) with erythrophagocytosis by blast cells images. Immunophenotyping confirmed T-cell LAL. Cytogenetic analysis was normal. Erythrophagocytosis by blast cells in patients with T-cell LAL appears to be a separate entity, hence the importance of images on diagnosis, prognosis and treatment of T-cell LAL.

[A monocentric study on the management of patients with myelodysplastic syndromes in Morocco]. / Prise en charge des syndromes myélodysplasiques au Maroc à propos d´une étude mono-centrique.

We conducted a retrospective descriptive analytical study in the Department of Clinical Haematology at the Mohammed V Military Training Hospital in Rabat over a period of 10 years. This study included 76 patients diagnosed with myelodysplastic syndrome (MDS) between 2008 and 2018. The average number of cases per year was 7.6. Out of 76 patients, 57% were men and 43% were women. The average age of our study population was 65.75 ± 12.55. The average age was 66.88 ± 13.10. No cases of profession exposed to disease was reported. Ninety-seven point three percent of patients had primary myelodysplastic syndrome and only 2 or 2.7% had myelodysplastic syndrome secondary to chemotherapy. The average time between the first visit and the diagnosis of myelodysplastic syndrome was, on average, 33.6 days ± 51, with a median of 19 days. The IPSS prognostic score was: low risk in 37.4% of cases, intermediate risk 1 in 46.6% of cases, intermediate risk in 12% of cases and high risk in 4% of cases. Thus, 84% of patients had low-risk MDS and 16% had high-risk MDS. Regular monitoring of patients showed many complications such as bleeding in 13% of patients, infections in 8% of cases, secondary hemochromatosis as a result of iterative transfusions in 6.6% of patients and transformation to acute myeloid leukemia in 2.7% of patients. In our study, abstention was the therapeutic choice in 42.1% of patients, transfusion was recommended in 35.5% of patients: red cells in 70% of cases, platelet concentrates in 40% of cases, iron chelators in 25% of transfused patients and EPO in 27% of patients. azacitidine was prescribed in 18% of patients, 50% had low-risk MDS and 50% had high-risk MDS. Bone marrow transplant was the only curative treatment for MDS. It was performed in a single patient with high risk MDS.

[Chronic paraneoplastic inflammatory demyelinating polyradiculoneuritis secondary to nasal natural killer lymphoma]. / Une polyradiculonévrite inflammatoire démyélinisante chronique paranéoplasique secondaire à un lymphome natural killer nasal.

We here report a single case of chronic paraneoplastic inflammatory demyelinating polyradiculoneuritis secondary to a nasal natural killer (NK) non-Hodgkin T lymphoma.

Prevalence and risk factors for latent tuberculosis infection among healthcare workers in Morocco.

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.

Effect of CYP2C9, VKORC1, CYP4F2, and GGCX gene variants and patient characteristics on acenocoumarol maintenance dose: Proposal for a dosing algorithm for Moroccan patients.

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 G>A, VKORC1 1173 C>T, CYP2C9*2, CYP2C9*3, CYP4F2 1347 G>A and GGCX 12970 C>G 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-value > 0.05). Non-parametric analysis confirmed the accumulate effect of variant alleles at VKORC1 -1639 G>A, VKORC1 1173 C>T 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.

Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia.

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.

Successful management of synchronous recurrent breast carcinoma with chronic myelogenous leukemia: a case report.

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.

[Primary plasma cell leukemia: about 3 cases]. / Leucémie à plasmocytes primitive: à propos de 03 cas.

Plasma cell leukemia is a rare hematologic malignancy defined by the presence of more than 20% of leukocyte count plasma cells or by a number of circulating plasma cells greater than 2 × 109/L (2G/L). It may be primary in 60% of cases and it manifests itself immediately as a leukemia or as a secondary event, in 40% of cases, complicating a previously diagnosed multiple myeloma. Given the rarity of this condition, only a few cases have been reported in the literature. It is characterized by its aggressiveness and poor prognosis. This study reports 3 cases diagnosed in the hematology laboratory of Mohamed V Military Hospital and aims to describe the clinical, biological and prognostic features of this disease.

Severe hemolytic disease of the premature newborn due to RH1 incompatibility: a case report.

We report a case of dramatic outcome of severe hemolytic 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.

Hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma.

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.

[A hemophagocytic syndrome revealing a Griscelli syndrome type 2]. / Un syndrome d'activation macrophagique révélant un syndrome de Griscelli de type 2.

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.

[Plasma cell leukemia: three case-reports and review of literature]. / La leucémie à plasmocytes : à propos de trois cas et revue de la littérature.

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.