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.

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%.

[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.

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.

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.

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.

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.

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.

[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.

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.

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.

[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.

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.