Non-Secretory Multiple Myeloma: A New Observation and Review of the Literature.

Non-secreting multiple myeloma is a rare variant of multiple myeloma that affects a relatively young population. It is characterized by the non-secretory nature of malignant plasma cells. The following case report describes the history of a 54-year-old patient with non-secretory myeloma revealed by mechanical and inflammatory low back pain. The bone and neurological involvement, the presence of diffuse osteolytic lesions and the increase in the serum kappa free light chains (FLC) level prompted a myelogram. This finds medullary invasion by dystrophic plasma cells. The absence of detection of complete or incomplete monoclonal immunoglobulin in the blood and urine, as well as the revelation of cytogenetic abnormalities of plasma cells, allows the diagnosis of non-secreting multiple myeloma to be made. This clinical case aims to describe the unusual presentation of this rare form of multiple myeloma.

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%.

The First Case of Hemoglobin J-Guantanamo Reported During HbA1c Measurement by High-Performance Liquid Chromatography at Moroccan Military Hospital Mohammed V.

We present a 57-year-old woman with diabetes mellitus and no other known comorbidities. HbA1c measurement by high-performance liquid chromatography (HPLC) gave unquantified results and a supernumerary peak was detected on the chromatogram. A thorough exploration of the hemoglobin profile showed the presence of an unclassified variant. Alkaline pH capillary electrophoresis revealed the presence of an abnormal peak migrating at zone 11, comprising 40.1% of total hemoglobin. An abnormal band migrating between hemoglobin A and hemoglobin F was observed by acid gel electrophoresis. Sequencing of the ß-globin gene confirmed the presence of a rare hemoglobin variant, hemoglobin J-Guantanamo (HBB:c.386C>A) in the heterozygous state, which was for the first time documented in Morocco. Through this report, we emphasize the importance of careful analysis of the HPLC chromatogram for the detection of possible hemoglobin variants in HbA1c measurement.

Influence of homocysteine and its major genetic and nutritional determinants on bone mineral density.

INTRODUCTION: A potential role of hyperhomocysteinemia in bone metabolism has been considered from the observation of high prevalence of osteoporosis in subjects with homocystinuria about 50 years ago. AIM: To examine the association of homocysteine level and its determinants Methylenetetrahydrofolate Reductase [MTHFR] C677T Polymorphism, folates and vitamin B12 levels with bone mineral density [BMD] and the prevalence of vertebral fractures [VF] on postmenopausal women. METHODS: Through a cross-sectional study, one hundred and twenty-two healthy postmenopausal women gave their informed consent to participate in this study. Women were recruited through advertisements and mouth to ear between January 2017 and May 2017. One serum tube and one EDTA tube were collected from fasting patients. Bone mineral density was determined by a Lunar Prodigy® Vision DXA system®. Vertebral fracture [VF] assessment image was inspected visually by 2 clinicians. RESULTS: We found that a high level of homocysteine and low vitamin B12 and folate levels are not associated with bone mineral density and are not risk factors for VF in healthy postmenopausal women. Whereas, the presence of VF was associated with the number of years since menopause and with the osteocalcin levels. CONCLUSION: The MTHFR C677T polymorphism, the high levels of HCY, or low levels of folate and vitamin B12 would not be risk factors for osteoporosis and VF in healthy postmenopausal women.

[Association of hemoglobinosis D-Punjab and ß-thalassemia in a Moroccan family]. / Association d'hémoglobinose D-Punjab et de ß-thalassémie dans une famille marocaine.

Hemoglobin D-Punjab is a common hemoglobin variant in India but very rare in Morocco. Often, its presence has minimal or no clinical impact. Its heterozygous association with ß-thalassemia is exceptional. The purpose of the study is to describe the epidemiological, diagnostic and prophylactic aspects of hemoglobinosis D-Punjab from a family case study. MATERIAL AND METHODS: Case study of hemoglobinosis D-Punjab in a Moroccan family, diagnosed at the Laboratory of Biochemistry-Toxicology of the Mohammed V Military Teaching Hospital. The biological study was based on iron and hemolysis checkups, hemogram and study of hemoglobin (electrophoresis in alkaline and acid medium, high performance liquid chromatography). The index patient also benefited from sequencing by molecular biology. RESULTS: The index patient was heterozygous D-Punjab/ß0-thalassemia, confirmed by molecular biology. Two of her sisters had the same hemoglobin profile. At electrophoresis, all three had hemoglobin D-Punjab higher than 90%, hemoglobin A less than 1% and hemoglobin A2 higher than 6%. The results of the three hemograms showed similar abnormalities (pseudo-polycythemia, hypochromia, microcytosis, anisopoikilocytosis). Six other members of the family had a thalassemic trait and another three had heterozygous hemoglobinosis D-Punjab. CONCLUSION: Hemoglobin D-Punjab remains extremely rare in Morocco and very poorly documented in the literature. The number of reported cases is expected to raise due to increasing migration. Biologist advisory services require a precise diagnosis in order to give correct genetic counseling.

Interference Rendering Capillary Electrophoresis of Serum Proteins Uninterpretable.

BACKGROUND: The authors report a clinical case where biological interference rendered the electrophoretic trace of serum proteins obtained by capillary electrophoresis as uninterpretable. METHODS: Electrophoresis of serum proteins and immunotyping immunoassay were performed on Capillary 2 Flex Piercing® Sebia. RESULTS: A 72-year-old male patient was hospitalized in the clinical hematology department for deep lymphadenopathies with hepatocellular failure. The biological assessment revealed chronic renal failure stage G3b, icteric cholestasis with alkaline phosphatase levels, and hepatic cytolysis. Electrophoresis of serum proteins showed a thickening at the base of albumin peak on its anodic slope but, in particular, it revealed the appearance of a super-numerary split peak between albumin and α1 globulins. These peaks made it impossible to identify and integrate peaks. Based on the clinical information, immunotyping immunoassay was performed on the same sample. The comparison of the different curves with the reference curve does not show any monoclonal immunoglobulinopathy but eliminates all the supernumerary peaks. Suspecting an interference, a Hydrasys 2 Scan® agarose gel electrophoresis (Sebia) was run on the same sample; however, it did not show qualitative anomalies interfering with identification and integration of all the peaks. CONCLUSIONS: Our clinical case emphasizes the importance of the knowledge of certain endogenous interferences that may be the origin of unusual images when interpreting electrophoretic profiles, especially in capillary electrophoresis.

[Positivity threshold value for cardiac troponin lc in the diagnosis of perioperative myocardial infarction after on-pump cardiac surgery in adult patients]. / Seuil de positivité de la troponine i cardiaque dans le diagnostic de l'IDM péri-opératoire après chirurgie cardiaque sous circulation extra-corporelle chez l'adulte.

INTRODUCTION: This prospective study was conducted at the Laboratoy of Biochemistry of the HMIMV, Rabat, over a period of 1 year. It aimed to analyse the kinetics of troponin lc (cTnl) after on-pump cardiac surgery in order to establish threshold values for the diagnosis of perioperative myocardial infarction. METHODS: The study included 58 patients who had undergone on-pump valvular surgery or on-pump coronary artery bypass surgery. These patients were separated into 3 groups according to their clinical and electrical evolution as well as their laboratory tests (cTnI) during the immediate post-operative period. We batched and followed the kinetics of cTnl using immuno-enzymatic technique, CTNI Flex® reagent, before and after on-pump cardiac surgery, at H0, H3, H6, H12, H21, H24 and H72 using automatic SIEMENS Dimension Xpand Plus. RESULTS: The results were higher than in cardiology, even among the group of patients without postoperative cardiac complications, with an average cTnl rate of approximately 2.5 times higher than the threshold value in cardiology. The kinetics of release differed significantly among the 3 groups (p< 0.05). CONCLUSION: The threshold value that we propose to confirm the diagnosis of perioperative myocardial infarction is 13ng/ml obtained between H12 and H24. We recommend to collect 1-2 samples at H12 and then between H20 and H24.

Unexpected discovery of multiple myeloma following cardiomyopathy.

We report the case of multiple myeloma of unexpected discovery in an old patient admitted to the emergency department of cardiomyopathy. This observation emphasizes the need for exploring any anemia before linking it to heart failure or kidney disease. Serum protein electrophoresis remains crucial especially in the elderly patients.

A clinical picture of pulmonary embolism revealing light-chain myeloma.

We are highlighting on the particularity of a clinical picture of pulmonary embolism revealing light-chain myeloma in a 56-year-old male patient. Myeloma remains a rare affection. Even though its revelation through pulmonary embolism remains rare, it can be explained by hyperviscosity syndrome accompanying it.

[Fortuitous detection of composite heterozygous S/C sickle cell disease]. / Découverte fortuite d'une drépanocytose hétérozygote composite S/C.

Composite S/C sickle cell disease accounts for 20%-30% of major sickle cell syndromes. We report a case of fortuitous detection of composite heterozygous S/C sickle cell disease in the context of retinal detachment. The patient had been hospitalized in the Department of Ophthalmology for treatment-resistant decreased visual acuity detected 06 months before. The patient's clinical history was marked by total hip replacement (THR) twelve years before. Our study highlights the wide clinical variability of sickle cell disease underlying the importance of early screening and adapted clinical monitoring of patients at-risk, in order to avoid its evolution toward irreversible organic sequelae such as sickle cell retinopathy.

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.

Vitamin D status in healthy Moroccan men and women aged 50 years and older: a cross-sectional study.

UNLABELLED: This study aimed to compare the vitamin D status in healthy Moroccan men and women aged 50 years and older. A total of 186 Moroccan women and 68 men, who had no previous diagnosis of osteoporosis, were recruited prospectively. We found in this study a high prevalence of hypovitaminosis D with no difference between men and women. PURPOSE: The main purpose of this study was to describe and compare the vitamin D status, parathormone, calcium, and phosphate of healthy Moroccan men and women aged 50 years and older. METHODS: We conducted two cross-sectional studies, in postmenopausal women from October 2008 to November 2009 and in men over 50 years old, from December 2009 to August 2010. A total of 186 Moroccan women and 68 men, who had no previous diagnosis of osteoporosis, were recruited prospectively. For the definition of hypovitaminosis D, the preferred level for 25-hydroxyvitamin D (25(OH)D) insufficiency, which is now recommended by many experts, is 30 ng/mL (75 nmol/L), and the levels below 10 ng/ml (25 nmol/L) indicate deficiency. RESULTS: The prevalence of vitamin D deficiency in men and women was 4.4 and 8.6 %, respectively, and the prevalence of vitamin D(25(OH) D) insufficiency in men and women were 85.2 and 77.4 %, respectively. In men and women, no correlations were found between intact parathormone (PTHi) and 25(OH) D (r = 0.056). CONCLUSIONS: Despite a sunny environment, we found in this study a high prevalence of hypovitaminosis D (insufficiency + deficiency) in Moroccan men over 50 years old and postmenopausal women.

Relationships between vertebral fractures, sex hormones and vitamin D in Moroccan postmenopausal women: a cross sectional study.

BACKGROUND: Vertebral Fractures (VFs) are associated with bone loss that occurs before menopause but is accelerated at menopause as a result of sex hormone deficiency. To determine the association of sex hormones, bone remodeling markers and vitamin D levels with bone mineral density (BMD) and asymptomatic VFs prevalence using vertebral fracture assessment (VFA) in a cohort of Moroccan menopausal women. METHODS: This was a cross-sectional study conducted from October 2012 to April 2013 with menopausal women aged 50 years old and over. A total of 207 women who had no previous diagnosis of osteoporosis were enrolled in this cross-sectional study. Women were recruited prospectively from our laboratory department. VFA images and scans of the lumbar spine and proximal femur were obtained using a GE Healthcare Lunar Prodigy densitometer. VFs were defined using a combination of Genant semiquantitative approach and morphometry. Serum levels of estradiol, dehydroepiandrosterone sulfate, Sex hormone binding globulin, vitamin D, Osteocalcin, Crosslaps, intact parathormone were measured by Electrochemiluminescent immunoassay technique. RESULTS: Among the 207 women, 18.3 % (n = 38) had densitometric osteoporosis. On VFA, VFs were detected in 134 (62.3 %), including 96 (44.6 %) grade 1 and 38 (17.6 %) grade 2/3. There was no difference in the plasma levels of sex steroids, bone remodeling markers and vitamin D in the group of women with VFs (grade 1 and grade 2/3) and without VFs. The combination of variables that best predicted grade 2/3 VFs included the number of years since menopause and the lumbar spine T-score. CONCLUSION: These data confirm the importance of postmenopausal estrogen and SHBG concentrations in the bone loss and the pathogenesis of osteoporosis in elderly women, but not in the occurrence of the VFs.

[A case of bisalbuminemia in a patient with digestive adenocarcinoma]. / Un cas de bisalbuminémie chez une patiente avec adénocarcinome digestif.

UNLABELLED: Bisalbuminemia whether hereditary or acquired, is a rare electrophoretic abnormality of albumin, characterized by a duplication of the albumin fraction on the electrophoretic trace of the serum proteins. This duplication reflects the presence in the same individual normal plasma albumin and a modified albumin. OBSERVATION: This is a patient of 62 years hospitalized at the Internal Medicine Department of HMIMV for liver metastases of gastrointestinal adenocarcinoma and including serum protein electrophoresis (EP) performed on capillary (Capillarys society Sebia) reveals bisalbuminemia. The family investigation has ruled that inheritance of acquired bisalbuminemia to retain appearance. COMMENT: Bisalbuminemia acquired outside treatment with high doses of beta-lactam antibiotics, chronic pancreatitis with pseudocyst rupture or fixing a monoclonal immunoglobulin on albumin in the myeloma subjects is an exceptional event. The pathophysiologic mechanism in the reported cases remains unexplained as is also the case of acquired bisalbuminemias associated with other pathological contexts (Alzheimer's disease nephrotic syndrome). CONCLUSION: To the best of our knowledge, the bisalbuminemia in the gastrointestinal adenocarcinoma liver metastases has not been reported to date, hence the importance of the reported cases.

[Association of Kaposi's disease-immunoglobulin monoclonal: a new case]. / Association maladie de Kaposi et immunoglobuline monoclonale: un nouveau cas.

Kaposi disease, a tumor virus-induced, is a cutaneomucosis disease, generated by the virus infection HHV 8 of the gamma-Herpesviridae family. This virus is involved in several lymphoid pathologies. Its role in the plasma cell proliferation genesis during monoclonal gammopathy is discussed, and results are contradictory. The occurrence of Kaposi disease during multiple myeloma was described in the literature. Through this observation, we report the first case associated with monoclonal gammopathy, evolved for 3 years by HIV negative patient, and we discuss the involvement of HHV8 virus in the development of monoclonal immunoglobulin.

[Sensitization to aeroallergens at Mohamed V Hospital (Rabat, Morroco)]. / Étude des IgE réactives aux pneumallergènes chez les consultants de l'hôpital militaire Mohamed V de Rabat (Maroc).

This transversal case study aimed at drawing the biological profile of sensitized patients consulting for respiratory allergies at Mohamed V Military Hospital-Rabat. One hundred four patients were included in the case study, who have benefitted from specialized medical consultation and those who came to the biochemistry laboratory holding a medical check-up prescription for allergy (NFS and biochemical check-up). The biochemical analysis were made up of TMA Phadiatop(®), correct proportioning of specific IgE serum, and complete IgE. The different anamnestic and clinical parameters acting upon the sensitization to respiratory allergens have been collected through a multiple choice question survey. Statistical analysis has been performed by a SPSS software version 13.0. The Phadiatop(®) was positive at 54.8% of our patients with a masculine predominance (H/F sex-ratio = 1.4). The recurring symptoms were the rhinitis (36.8%) and the asthma (10.5%). The allergen d1 Dermatophagoides pteronyssinus was the most incriminated pneumallergens (59.6%). For 19.3% of the patients sensitized, a polysensitization (superior to three) was noted, due to dust mite, cockroaches, pollen and animals. 93.6% of the Phadiatop(®) positive patient had a hypereosinophilia (p < 0.001). The complete IgE had been prescribed to 29 patients and had been increased among 19 patients in 66% of the cases. Among the patients having a rate of elevated complete IgE, 8 (42.1%) had a negative Phadiatop(®) (p = 0.019). Our survey showed the strong prevalence of the respiratory sensitization at our population. The allergen d1 is the most incriminated. It also put in evidence the weak diagnostic value of the dosage of the complete IgE in the respiratory allergy.

[Multiple myeloma with D immunoglobulin]. / Myélome multiple à immunoglobuline D.

The immunoglobulin D multiple myeloma is a rare form of multiple myeloma and affects a young population. It is characterized by its clinical severity and poor prognosis. We report four cases of multiple myeloma immunoglobulin D diagnosed and supported in the university hospital Center of Sale and Rabat-Morocco. We propose to study the epidemiological, clinical and biological characteristics of this rare type of monoclonal gammopathy. Through the observations reported, the clinical aspect of myeloma is characterized by the high frequency of extra-bone manifestations including impaired kidney function. The immunoglobulin D multiple myeloma is mainly type λ, the IgD κ is rare, the predominance of λ light chains could be explained by rearrangements at the immunoglobulin genes. Bence-Jones proteinuria is almost constant in the multiple myeloma immunoglobulin D, it is mainly type λ, reflecting excess production of light chains by plasma cells. The marrow is invaded by plasma cells in very different proportions of up to 95%. It's a clinical entity, difficult to diagnose, particularly when low homogeneous band on electrophoresis goes unnoticed for an eye inexperienced or when immune serum anti-IgD was not used during the immunotyping.

[Lipid profile of patients on chronic hemodialysis (Morocco)]. / Profil lipidique dans l'insuffisance rénale chronique au stade d'hémodialyse : étude marocaine.

INTRODUCTION: Patients with end-stage renal disease (ESRD) receiving chronic hemodialysis show a high incidence and prevalence of cardiovascular disease of multifactorial etiology and an association between dyslipidemia and accelerated atherosclerosis. OBJECTIVE: Our aim was to study lipid profiles in ESRD patients receiving dialysis regularly at our hospital (Morocco).Subjects and methods : The patient population consisted of 30 ESRD patients on maintenance haemodialysis. Matched control subjects were recruited among healthy normolipidemic patients. Concentrations of triglycerides (TG), total cholesterol (TC), high-density-lipoprotein cholesterol (HDL-C) and low-density-lipoprotein cholesterol (LDL-C) were measured. The atherogenic index (AI = TC/HDL-C ratio) was calculated. RESULTS: The TG, the HDL-C levels and atherogenic index were significantly higher in groups of hemodialysis patients. We saw no increase in the levels of TC and LDL-C. The prevalence of dyslipidemia in hemodialysis group was high (80%). The most frequent lipid alterations were decreased HDL-C (70%), increased TG (33,3%) and increased LDL-C (23,3%); 50% of ESRD patients have more than two different dyslipidemic findings. AI was higher (≥ 5) in 33,3% of cases. CONCLUSION: The prevalence of dyslipidemia is higher than normal in ESRD patients on maintenance hemodialysis. Classically, these patients have had low levels of HDL-C and elevated TG levels. Strict control of dyslipidemia should be part of the cardiovascular risk prevention strategy in this population.