Graves' Disease: Acquired Cause of a Moderate Increase in Hemoglobin A2 Level.

BACKGROUND: Hyperthyroidism can lead to diverse hematological disorders, such as microcytosis and a mild increase in hemoglobin A2 fraction. METHODS: This study reported a 31-year-old woman of Moroccan origin recently diagnosed with Graves' disease. Her blood tests revealed microcytosis, hypochromia, and a normal ferritin level. A phenotypic analysis of hemo-globin was performed using two techniques: capillary electrophoresis and reversed-phase high performance liquid chromatography. RESULTS: Both techniques indicated a slight increase in hemoglobin A2 level. These results initially suggested het-erozygous beta-thalassemia, eventually correlating with the concurrent presence of Graves' disease, as evidenced by the normalization of hemoglobin A2 level following treatment. CONCLUSIONS: This case highlights the importance of having clinical, biological, and therapeutic data for a relevant interpretation of a phenotypic hemoglobin study.

Atypical Migration in Serum Immunofixation of Immunoglobulin A with Masked kappa Light Chains: a Case Report and Review of the Literature.

BACKGROUND: We report a case of a patient with immunoglobulin A multiple myeloma associated with a masked kappa light chain. Serum immunofixation showed a monoclonal band in the IgA heavy chain lane without corre-spondence with the light chain and a monoclonal band in total kappa light chain lane without correspondence with the heavy chain. METHODS: To distinguish between heavy chain disease and immunoglobulin with "masked" light chains, two tubes containing the patient's serum were incubated with a very high concentration of anti-total kappa and anti-total lambda antisera for 48 hours at 4°C in order to facilitate immunoprecipitation of the involved light chain. After centrifugation, the supernatant was analyzed by using the IFs method on the Hydrasys 2 Scan Focusing Sebia® without dilution. Then we applied the anti-IgA, anti-total kappa and anti-total lambda antisera. RESULTS: The serum immunofixation test of the sample treated with a high concentration of anti-total kappa showed the disappearance of the monoclonal bands corresponding to IgA heavy chain lane and kappa light chain lane, indicating that precipitation had occurred and that the IgA did have kappa light chains that could not be detected by the standard immunofixation protocol. The serum immunofixation test of the sample treated with anti-total lambda showed the disappearance of the polyclonal background in lambda light chain lane, confirming that the precipitation with lambda light chains according to the previously mentioned protocol has done well. CONCLUSIONS: This case illustrates some of the difficulties encountered and the corrective actions that can be taken for the detection of immunoglobulins with masked light chains.

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%.

Monoclonal Immunoglobulin Associated with Monoclonal Free Light Chains Invisible on Capillary Electrophoresis.

BACKGROUND: We report the case of a monoclonal immunoglobulin of IgM Lambda isotype associated with monoclonal lambda-type free light chains not detected by capillary electrophoresis but identified by immunofixation. METHODS: Capillary electrophoresis showed hypoproteinemia and an inflammatory syndrome. The IF realized on Hydrasys 2 Scan Focusing Sebia® reveals an IgM monoclonal band and two monoclonal bands in the total lambda. A second IF is performed using anti IgM, anti IgD, anti IgE and anti-total and -free lambda light chains as antisera. It reveals the presence of a monoclonal protein isotype IgM Lambda with free light chains. In view of these discordant results, an immunosubtraction was performed on the same sample showing no abnormality. RESULTS: Our patient has a monoclonal IgM Lambda with lambda monoclonal free light chains all masked on capillary electrophoresis and therefore not detected. CONCLUSIONS: Capillary electrophoresis techniques are incrementally becoming the techniques of choice in medical laboratories as a replacement for gel electrophoresis, due to their automation and better sensitivity. However, in some cases, a monoclonal immunoglobulin may not be detected by capillary technique and may cause an inaccurate interpretation.

Pseudo-Hyperchloremia: Think about Analytical Interference with Bromides.

BACKGROUND: We report a case of pseudo-hyperchloremia in a patient hospitalized in the dermatology department at the Mohammed V Military Hospital of Instruction in Rabat. The examination revealed self-medication with calcium bromo-galactogluconate, allowing the suspicion of an analytical interference on chloremia by bromides. METHODS: The determination of chloremia was done by indirect potentiometry on an Architect ci8200®. RESULTS: A biological assessment was carried out on admission, showed isolated hyperchloremia at 137 mmol/L (95 - 110 mmol/L), while the other electrolytes, the anion gap, and kidney function were normal. A follow-up electrolytes test one week after stopping calcium bromo-galactogluconate showed normalization of chloremia to 109 mmol/L. CONCLUSIONS: The analytical interference of bromides on the chloride assay causes pseudo-hyperchloremia, an analytical anomaly not very well-known by clinicians.

A Polymerized Kappa Light Chain Multiple Myeloma.

BACKGROUND: We report a case of a multiple myeloma with light polymerized kappa chains, a phenomenon rarely described. METHODS: Capillary electrophoresis of serum proteins (Capillarys 2 Flex Piercing Sebia ®) revealed the presence of two migrant monoclonal peaks in the gamma globulin area and identified by immunosubtraction (IS) as light chain (LC) κ. These results suggest either the presence of a LCκ monoclonal IgD or IgE or the presence of a biclonal gammopathy or finally a LCκ polymerization. Serum immunofixation (IF) was carried out, after serum depolymerization with beta mercaptoethanol (BME), using as antiserum anti IgD, anti IgE and anti LCκ total and free LC κ. Capillary electrophoresis and IS were also repeated after serum treatment with BME. RESULTS: The depolymerization of our patient's serum showed the transformation of the two serum peaks into a single peak at the level of the κ light chains on both capillary electrophoresis and immunotyping techniques (IF and IS). CONCLUSIONS: Polymerization of kappa light chains is a rare phenomenon which can cause difficulty in the interpretation of the serum protein electrophoresis and immunotyping.

[>Femoral bone infarction revealing compound heterozygous SC sickle cell disease in a Moroccan patient]. / Infarctus de l´os fémoral révélant une drépanocytose composite SC chez un patient marocain.

Double heterozygosity SC is a major sickle cell syndrome. Its course may be marked by severe or irreversible complications, such as bone infarction. We here report the case of a 17-year-old patient presenting with compound heterozygous SC sickle cell disease following severe knee pain (gonalgia) in order to highlight the risk of delayed diagnosis as well as the need for prevention policies guiding early screening, thus improving patients' management and prognosis.

Fortuitous Discovery of Hereditary Xanthinuria.

Hereditary xanthinuria was the first inherited purine metabolism disorder described. It is a rare pathology, which is most often asymptomatic and whose incidence is therefore underestimated. We report the case of a patient with an undetectable level of uric acid in the blood, discovered during a systematic biological assessment. This case report recalls the existence of this rare metabolic disorder, which is usually benign, but can lead to complications, and the importance of considering an abnormality of the purine cycle when discovering a hypo-uricemia.

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.

Unexpected discovery of hemoglobinopathy C/ß° thalassemia.

High performance liquid chromatography (HPLC) is the current method of choice for the detection of hemoglobinopathies and the quantification of A2 and fetal hemoglobin. We are describing a case where a double heterozygosity C/beta-thalassemia was fortuitously identified, during assaying HBA1c, by HPLC.

[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.

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:l'objectif de cette étude prospective, réalisée sur une année au laboratoire de biochimie de l'HMIMV de Rabat, vise à étudier la cinétique de la Troponine Ic (TnIc) après chirurgie cardiaque sous circulation extracorporelle (CEC) en vue d'établir des valeurs seuils pour le diagnostic d'infarctus de myocarde péri-opératoire (IDMPO). Méthodes: nous avons inclus 58 patients opérés pour chirurgie valvulaire ou pontage coronarien sous CEC. Ces patients ont été séparés en 3 groupes selon l'évolution clinique, biologique (TnIc) et électrique durant la période post opératoire précoce. Nous avons dosé et suivi la cinétique de la TnIc par une technique immuno-enzymatique, au moyen du réactif FlexR CTNI avant et après la CEC, à H0, H3, H6, H12, H21, H24 et H72 sur l'automate Dimension Xpand plusR de la société Siemens.Résultats: les résultats obtenus sont plus élevés qu'en cardiologie, même chez le groupe des patients sans complications cardiaques post opératoires, avec un taux moyen de TnIc environ 2,5 fois supérieur à la valeur seuil en cardiologie. La cinétique de libération est significativement différente entre les 3 groupes (p<0,05).Conclusion: la valeur seuil que nous proposons pour confirmer le diagnostic de l'IDMPO est de 13ng/ml obtenue entre H12 et H24. Nous recommandons, de ce fait, un à deux prélèvements vers H12 puis entre H20 et H24

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.