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.

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.

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.

A Rare Coexistence of Smoldering Multiple Myeloma and JAK2-Positive Myeloproliferative Neoplasm: A Case of Dual Synchronous Hematological Malignancy.

This article explores the rare case of an 82-year-old man diagnosed concurrently with essential thrombocythemia and smoldering multiple myeloma (SMM). The limited existing literature on individuals harboring both myeloproliferative neoplasm (MPN) and monoclonal gammopathy (MG) is of significant interest due to the distinct origins of these malignancies. The etiology of MG in MPN patients remains elusive, leading to speculation about a potential relationship or interplay between the two conditions. This unique case prompts a deeper exploration of the mechanisms underlying the coexistence of JAK2-positive MPN and SMM. It underscores the importance of tailored therapeutic strategies that carefully consider the inherent risks and potential adverse outcomes associated with these specific malignancies, thereby warranting further clinical research.

Soft Tissue Mucosa-Associated Lymphoid Tissue Lymphoma: A Rare Case With an Interesting Presentation and Review of the Literature.

Soft tissue mucosa-associated lymphoid tissue (MALT) lymphoma is a rare type of marginal zone lymphoma. Herein, we report a case of a 61-year-old patient who developed soft-tissue marginal zone lymphoma in the right arm. He was treated with rituximab-chlorambucil with good metabolic response and no evidence of disease recurrence after one year of follow-up.

Fatal Cryptococcal Meningitis in a Patient With Chronic Lymphocytic Leukemia Treated With Ibrutinib.

According to the latest World Health Organization classification published in 2022, chronic lymphocytic leukemia (CLL) is classified as a low-grade proliferation of clonal B-cells. The Bruton tyrosine kinase (BTK) pathway plays a crucial role in B-cell receptor signaling. Ibrutinib, the first irreversible BTK inhibitor, has been shown to improve the survival of CLL patients with lower toxicity than traditional chemotherapy. Cryptococcosis is an invasive fungal infection that primarily affects individuals with compromised immune systems. We present a case of a 69-year-old male with relapsed CLL who received treatment with ibrutinib and subsequently developed meningeal cryptococcosis, presenting with seizures and fever. A physical exam showed bilateral hypoacusis, but no focal deficits. Cerebral imaging was normal and laboratory results showed a low gamma globulin level and leucopenia with lymphopenia but without neutropenia. The cerebrospinal fluid profile was not inflammatory, opening pressure was normal, the classic India ink test was positive, and fungal cultures grew Cryptococcus neoformans. To complete investigations, HIV testing was negative, and sinus and chest tomography scans showed no anomalies. Treatment consisted of discontinuing ibrutinib and administering anti-fungal therapy with liposomal amphotericin (4 mg/kg/day) in combination with flucytosine (25 mg/kg/day). However, the patient's neurological status declined, and he passed away. This case highlights the potential risk of developing opportunistic infections such as cryptococcal meningitis in CLL patients treated with ibrutinib. It is crucial to consider the patient's immune status when administering ibrutinib and to closely monitor for signs of infection.

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%.

[Primary granulocytic sarcoma of the peritoneum: a case report and literature review]. / Sarcome granulocytaire à localisation péritonéale primitive : à propos d'un cas et revue de la littérature.

Granulocytic sarcoma is a rare tumor composed of immature granulocytic cells. Prognosis is poor. The periosteum is preferentially involved. A peritoneum localization is unusual. We report the case of a 20 years old man without particular previous pathologies, which brutally presented an ascitic syndrome in a context of health impairment state. The laparoscopy showes many white nodules on all the peritoneum. The histologic examination of one of these nodules showed granulocytic sarcoma. The blood and bone marrow cell count are without any anomaly. The treatment consisted of a standard acute myeloid leukaemia's chemotherapy with very good evolution. The rarity of peritoneal chloroma causes a diagnostic problem, especially in the absence of hematologic abnormalities. It must be mentioned in the presence of peritoneal nodules even if the blood count and bone marrow are normal.

Posterior Reversible Encephalopathy Syndrome Secondary to R-CHOP Chemotherapy Regimen.

Chemotherapy-induced posterior reversible encephalopathy (PRES) syndrome is a rare event. Its recurrence after reusing the incriminated molecules remains unpredictable. We report the case of a 58-year-old female patient being followed for a diffuse large B-cell lymphoma treated with rituximab, cyclophosphamide, hydroxydaunorubicin hydrochloride (doxorubicin hydrochloride), vincristine (Oncovin), and prednisone (R-CHOP) regimen. On the fourth day of the first R-CHOP cycle, the patient suddenly developed a headache, bilateral blurred vision, and drowsiness. The next day (day five), the patient had a spontaneously-resolving generalized tonic-clonic seizure associated with postictal bilateral blindness without any other neurological deficiency. Brain magnetic resonance imaging (MRI) revealed an increased bilateral signal intensity involving the cortex and subcortical white matter of the parietal and occipital lobes on the T2-weighted and the T2-weighted fluid-attenuated inversion recovery (FLAIR), which confirmed the diagnosis of PRES) syndrome. After resolution of symptoms, the continuation of the R-CHOP regimen did not lead to a recurrence of the syndrome.

[Erythroblastopenia and primary myelofibrosis: a very rare association (a case report)]. / L'érythroblastopénie et la myélofibrose primitive: association très rare (à propos d'un cas).

The combination of erythroblastopenia and primary myelofibrosis is very rare. We here report the unusual case of a 76-year-old Moroccan patient followed up since 2018 for idiopathic erythroblastopenia, initially treated with corticotherapy and then with ciclosporin. Two years later, the patient reported bone pain with splenomegaly. Assessment including myelogram, bone marrow biopsy and molecular biology showed myelofibrosis. Etiological assessment of myelofibrosis was negative confirming its primitive nature. The patient received ruxolitinib with transfusion support. Patient´s outcome was favorable and marked by improvement of general condition, splenomegaly and transfusion rate. The association between erythroblastopenia and myeloproliferative disorder is exceptional and only a few cases have been reported in the literature.

Bleomycin-Induced Lung Toxicity in Hodgkin's Lymphoma: Risk Factors in the Positron Emission Tomography Era.

Introduction Bleomycin is a major antimitotic agent in the first-line treatment for Hodgkin's lymphoma. The main limitation of its use is its pulmonary toxicity. The objectives of this study are to find out the risk factors for the occurrence of bleomycin-induced lung toxicity in patients with Hodgkin's lymphoma and, on the other hand, to determine if positron emission tomography scan is a reliable means of early detection of this toxicity. Methods This is a retrospective study conducted in the clinical Hematology Department of Mohammed V Military Instruction Hospital, Rabat, Morocco. All patients with Hodgkin's lymphoma and treated with a bleomycin-based chemotherapy were included. The impact of different clinical and biological factors on the risk of bleomycin-induced lung toxicity occurrence was assessed using univariate and multivariate logistic regression. The benefit of positron emission tomography, usually performed as part of the re-assessment of Hodgkin's lymphoma after two and four cycles, has been evaluated in the detection of bleomycin-induced lung toxicity. Results Among 124 patients included in the study, 18 (14.5%) patients experienced bleomycin-induced lung toxicity. On multivariate analysis, smoking (p = 0.038) and the use of the ABVD regimen (doxorubicin, bleomycin, vinblastine, and dacarbazine) compared to the escalated BEACOPPe regimen (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) (p = 0.018) were statistically significant risk factors. After two and four courses of therapy, the positron emission tomography was able to predict the occurrence of bleomycin-induced lung toxicity before the appearance of clinical symptoms only in 36.4 % and 12.5% of patients, respectively. Conclusion Studies to identify risk factors for the development of bleomycin-induced lung toxicity are crucial to reduce toxicity in the treatment of Hodgkin's lymphoma. However, two- and four-cycle positron emission tomography scans cannot be considered as a reliable means of early detection of this toxicity.

Essential thrombocythemia and aortic dissection,causal or incidental association?

Only few cases of vascular dissection and essential thrombocythemia association have been reported. To the best of our knowledge, we reported the second case of aortic dissection and essential thrombocythemia association in a 60-year-old man with positive JAK2V617F mutation who had no history of hypertension or connective tissue disorders. Through this case, we discussed the eventual existence of a causal relationship between the two conditions. We also suggested the use of hydroxyurea as a prevention treatment of thrombosis in myeloproliferative neoplasms.

[Candida famata candidemia in immunosuppressed patient: report of a case with literature review]. / Candidémie à Candida famata chez un patient immunodéprimé: à propos d'un cas et revue de littérature.

Candida Famata is an opportunistic pathogen. Very rare cases of candidemia Candida Famata have been described. We report a case of a patient of 16 years undergoing chemotherapy for Hodgkin's disease. The patient was put under voriconazole for 3 weeks with a good evolution. This case confirms the opportunistic pathogen Candida Famata. All insulation in the blood must be considered before a background of immunosuppression.

Bilateral renal involvement in diffuse large B-cell lymphoma on fluorodeoxyglucose positron emission tomography/computed tomography.

Secondary renal involvement in patients with diffuse large B-cell lymphoma (DLBCL) is rare and associated with poor prognosis. We, hereby, described a case of a patient diagnosed with DLBCL, in whom bilateral renal involvement was detected on 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT). The patient received 4 courses of chemotherapy, and follow-up 18F-FDG PET/ CT revealed the complete resolution of the diffuse increased renal 18F-FDG uptake. Renal lymphoma is uncommon and 18F-FDG PET/CT is particularly useful for detecting extranodal involvement in DLBLC.

Splenic marginal zone lymphoma associated with hepatitis B virus infection, remission after viral treatment, and splenectomy: A case report and review of the literature.

We report the case of a patient diagnosed with a splenic marginal zone lymphoma with a simultaneous finding of hepatitis B virus infection, who responded to antiviral treatment and splenectomy. We highlighted this association described in the literature and its possible causal role, as well as the available therapeutic choices.

Hemophagocytic lymphohistiocytosis associated with HBV-HCV coinfection in adult: Case report.

The case reported is the first case in Morocco to our knowledge. The reason for sharing this case is to facilitate knowledge transfer between physicians, caring for adult patients with HLH, with the aim to improve the outcome of these patients.

Immature platelets: a review of the available evidence.

Immature platelets or reticulated platelets are newly released thrombocytes. They can be identified by their large size and high RNA cytoplasm concentration. Immature platelet fraction (IPF) represents the percentage of immature circulative platelets to the total number of platelets. The development of analytical standardization of this hematological parameter by new automated devices allowed a better exploration of its contribution in a context of thrombocytopenia. In fact, several studies had confirmed its clinical utility to differentiate immune thrombocytopenia from other causes of thrombocytopenia. IPF can also predict platelets recovery after chemotherapy and successful engraftment. In addition, immature platelets have shown utility in other diseases such as coronary artery diseases, bacterial infections and liver diseases. Despite all these advantages, immature platelet fraction can be increased in some cases of thrombocytopenia characterized by platelets hypoproduction. The aim of this review is to present the immature platelet fraction contribution in clinical practice.

Bing-Neel syndrome revealing Waldenström's macroglobulinemia: a case report.

INTRODUCTION: Bing-Neel syndrome is a lympho-plasmocytic infiltration of the central nervous system. The Bing-Neel syndrome is a rare entity, which often occurs during the evolution of a Waldenström's macroglobulinemia but can in some cases be the revealing mode of it. OBSERVATION: We report the case of a 56-year-old patient with tumoral form of Bing-Neel syndrome revealing Waldentrom's macroglobulinemia. CONCLUSION: This observation describes a rare entity whose diagnosis and therapeutic management is complex.

Acute Laryngeal Dyspnea as the First Presentation of Mantle Cell Lymphoma: A Case Report and Review of the Literature.

INTRODUCTION: Acute laryngeal dyspnea is a life-threatening emergency, and the causes in adults are most often laryngeal tumors or inflammatory edema. Lymphoma of the larynx and especially the mantle cell type is extremely rare. Case Presentation. We report a case of a 43-year-old woman with no particular pathological history. She presented with progressive dyspnea which has evolved towards an inspiratory bradypnea that worsened until she ultimately required an emergency tracheotomy. Biopsies revealed mantle cell lymphoma. The patient has been staged IVB MIPI 6, and she was treated by immunochemotherapy followed by ASCT. The therapeutic evaluation shows a complete remission, 18 months after, and the patient was always disease free. CONCLUSION: The laryngeal localization of the mantle cell lymphoma is extremely rare; it may present catastrophically with acute airway obstruction. The diagnosis is mostly histological, hence the interest of deep biopsy. Given its rarity, the therapeutic strategy must be discussed case by case in a multidisciplinary consultation meeting.