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.

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.

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.

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.

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.

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.

A rare case of perimyocarditis induced by all-trans retinoic acid administration during induction treatment of acute promyelocytic leukemia.

Treatment by All-Trans Retinoic Acid (ATRA) combined with an Anthracycline Chemotherapy has been shown to improve disease-free survival in patients with Acute Promyelocytic Leukemia (APL). Although ATRA is generally well tolerated, occurrence of Retinoic Acid Syndrome (RAS) during the induction chemotherapy in some patients is recognized as a distinct complication and a potential life-threatening side effect. Isolated myocarditis as a result of RAS related to ATRA administration is uncommon and has been rarely reported in the literature. We report a very rare case of ATRA-induced perimyocarditis accompanied by hemodynamic compromise. There was complete resolution of the signs and symptoms of peri-myocarditis when ATRA was temporary suspended. Finally, ATRA was safely resumed.

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.

Primary Burkitt lymphoma of the thyroid gland: case report of an exceptional type of thyroid neoplasm and review of the literature.

BACKGROUND: Primary thyroid lymphoma is an uncommon pathological entity that accounts for only 1 to 5 % of all thyroid malignancies. Primary Burkitt lymphoma of the thyroid gland is very rare. This article presents the first Moroccan case of a primary BL of the thyroid to be reported in the literature to date. CASE PRESENTATION: We describe here a case of a 70-year-old male who developed a rapidly enlarging thyroid gland with progressive symptoms of compression. Core biopsy confirmed the diagnosis of Burkitt lymphoma. The patient died of septic shock, 2 weeks after the first cycle of appropriate therapeutic chemotherapy. CONCLUSIONS: This presentation emphasizes the importance of considering lymphoma when dealing with a thyroid mass, as its management is different from that of other thyroid pathologies, and affords an opportunity to review a very rare type of primary thyroid lymphoma.

[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.

[Association between multiple myeloma and acute myeloid leukemia secondary to myelodysplastic syndrome]. / Association d'un myélome multiple et d'une leucémie aiguë myéloïde secondaire à un syndrome myélodysplasique.

We report a rare case of association of two distinct hematologic malignancies: refractory cytopenia with multilineage dysplasia associated with del(5q) and symptomatic multiple myeloma associated with del(17p) and del(13q). After 16 months, the patient presented an acute leukemic transformation of the myelodysplasic syndrome.

[Light-chain escape from plateau phase]. / Échappement des chaînes légères de la phase de plateau.

We report the first case of light-chain escape from plateau phase occurring after 7 years of evolution of multiple myeloma in a patient who had never received new molecules. A very good partial response was obtained.

[Signification of Epstein-Barr virus detection in the cerebrospinal fluid of a patient with human immunodeficiency virus related Burkitt lymphoma]. / Signification de la détection du virus d'Epstein-Barr dans le liquide céphalorachidien d'un patient suivi pour un lymphome de Burkitt lié au virus de l'immunodéficience humaine.

The significance of Epstein-Barr virus detection in the cerebrospinal fluid of patients with Burkitt lymphoma is poorly studied. We report the case of a patient with immunodeficiency associated Burkitt lymphoma in complete remission who presented 5 months after the end of treatment, an isolated optic neuritis. Lumbar puncture found lymphocytic meningitis and the viral load of Epstein-Barr virus was 234,000 copies per milliliter in the cerebrospinal fluid. These symptoms could be explained by Epstein-Barr virus meningoencephalitis but the detection of MYC rearrangements in the cerebrospinal fluid confirms the diagnosis of Burkitt lymphoma cerebral relapse. The detection of the Epstein-Barr virus DNA in the cerebrospinal fluid should be interpreted with caution.