Anaplastic Large-cell Lymphoma in Children: State of the Art in 2023.

Anaplastic large-cell lymphoma is a rare disease and account for approximately 10% to 15% of pediatric non-Hodgkin lymphomas. They are characterized by extended stages, a high frequency of B signs and extra nodal involvement. Multiagent chemotherapy cures ∽60% to 75% of patients and relapse occurs in 35% of cases. For relapsed patients, various treatments ranging from vinblastine monotherapy to therapeutic intensification with hematopoietic stem cell transplantation have been evaluated, but there is currently no consensus on the optimal therapeutic strategy. New therapeutic perspectives are being evaluated for relapses and refractory forms as well as high-risk forms including monoclonal antibodies (Anti CD30), ALK inhibitors, and CART cells.

[Evaluation of training in transfusion safety offered to medical students in Morocco]. / Evaluation d´une formation en sécurité transfusionnelle auprès des étudiants en médecine au Maroc.

Introduction: students worldwide receive little training on blood transfusion therapies during their time at medical schools and hospitals, then reformulation of academic programs is mandatory. Indications for blood transfusions are more frequent than recommended, which helps to increase the risks associated with this procedure. To overcome this, structured objective training on nursing care and procedures was organised with the topic of transfusion safety. The purpose of our study was to assess medical students´ appreciation of this training and the degree to which this population had mastered it. Methods: we conducted a cross-sectional, descriptive, single-centre study. We conducted a survey among the 3rd-year medical students. A self-administered assessment questionnaire, as well as an evaluation grid for acquired skills, filled in by the training doctors during the session, were used. Data analysis was based on descriptive statistics using Excel software. Results: three hundred and eighty-four (n=384) students were invited to attend this training course, 275 (71.6%) of whom were enrolled in the study. The overall satisfaction rate was 93.8%. The objectives and organisation were 95.6% successful. The quality of the training was 90.3% satisfactory. The choice of topic for the station was satisfactory for 80% of participants, the flow of the session for 86.3%, the quality of the organisation and debriefing for 89%, the interaction between trainers and learners for 90.2%, the motivation of trainers and the reflection stimulated in the learners for 92%. We noted that 93% of students had never participated in training in transfusion safety. Mastery of global skills was total for 67%, partial for 26% and absent for 7%. The students mastered the procedures for checking the identity and grouping of the product to be transfused at 97%, the principle of interpreting the ultimate pre-transfusion test (96%), the purpose of ultimate test at the patient´s bedside 93% with mastery of its technical implementation 83%. Conclusion: training in transfusion safety was well received, with a satisfactory level of proficiency. This experience can easily be extended to other topics.

An unusual presentation of subcutaneous panniculitis-like T-cell lymphoma: Extensive necrosis and hemophagocytic lymphohistiocytosis: A case report.

Key Clinical message: Subcutaneous panniculitis-like T-cell lymphoma, a primary cutaneous lymphoma, which is described as following a slow course, could claim life. The occurrence of facial and breast nodules, the association with hemophagocytic lymphohistiocytosis, and the extent of necrosis and ulceration are signs of its aggressive nature needing early diagnosis and prompt treatment. Abstract: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare skin disease that accounts for <1% of all peripheral T-cell lymphomas. It is described as following a slow and gradual process. However, it can be associated with a variety of clinical symptoms ranging from mild to severe. Hemophagocytic lymphohistiocytosis (HLH), a rare and potentially fatal hematologic factor that complicates SPTCL in 20% of cases, is an important prognostic factor. We report here an aggressive case of disseminated SPTCL with HLH involving a young woman who presented with extensive necrosis and ulceration at diagnosis. The report highlights the aggressive course of the disease, the occurrence of facial and breast nodules, the association with HLH, and the extent of necrosis and ulceration. The report highlights the poor prognosis despite polychemotherapy regimen use.

Epidemiological, clinical and therapeutic profiles of mantle cell lymphoma cared for in a Moroccan center: a review of 14 cases.

Mantle cell lymphoma (MCL) accounts for 3-10% of non-Hodgkin's lymphomas (NHL). We identified 14 patients with mantle cell lymphoma, with an average number of 3.5 new cases/year. A male predominance was observed with a sex ratio equal to 6. The average age of our patients was 64.4±14.1 years, with an average diagnostic delay of 6.57 months. Regarding the clinical presentation, adenopathy was the most reported physical sign (78.6%) followed by B symptoms (57.1%). Disseminated stages were the most frequent in our series: stages IV (78.5%) and III (7.1%) versus stages I (0%) and II (7.1%). The extra-ganglionic localizations observed were hepatic 5 cases (31.1%), pulmonary 04 cases (25%), medullary 4 cases (25%), pleural 2 cases (12.5%) and prostate 1 case (6.2%). All diagnosed cases are mantle cell lymphomas, of which 12 cases (85.7%) are classical and 2 cases (14.3%) indolent. The high-risk group is, according to international prognostic index (MIPI) MCL prognostic score, the most represented in our series: 0-3 = 6 cases (42.9%), 6-11 = 8 cases (57.1%). The therapeutic protocol chosen 1st line: 9 patients treated with R-DHAP, three with R-CHOP, one with DHAOX and one with R-CVP. Second line: two patients treated with R-DHAP, one after R-CHOP and the other after R-CVP. Two patients received autologous hematopoietic stem cell transplant at the end of the treatment. The evolution was marked by the death of 7 patients, 3 lost to follow-up and 4 still followed. Additionally, the study highlights characteristics and treatment patterns of mantle cell lymphoma, emphasizing its predominance in males, delayed diagnosis, frequent dissemination, and high-risk classification, with chemotherapy as the primary treatment modality and a challenging prognosis contributing to a comprehensive understanding of mantle cell lymphoma presentation and management.

Prolonged SARS-CoV-2 infection during autologous hematopoietic stem cell transplantation from Hodgkin's lymphoma: a case report.

Autologous hematopoietic stem cell transplantation (HSCT) for relapsed Hodgkin's lymphoma increases the risk of infection by using intensive chemotherapy. This risk is obviously ongoing given the increased virulence of severe COVID-19. We report a case of a young man with Hodgkin's lymphoma who received conditioning chemotherapy followed by autologous HSCT and tested positive for SARS-CoV-2 by polymerase chain reaction (PCR) during the early phase of aplasia with persistence of COVID-19 beyond 30 days with favorable follow-up and clinical improvement on treatment. For this type of patient with hematologic malignancy, viral infection can be fatal and strict medical precautions with isolation rules must be maintained, especially for SARS-CoV-2.

[Cardiac tamponade as first manifestation of multiple myeloma : A case report with literature review]. / Tamponnade cardiaque : première manifestation du myélome multiple : à propos d'un cas et revue de la littérature.

Multiple myeloma is a hematologic malignancy characterized by clonal proliferation of plasma cells, mainly in bone marrow. Extramedullary disease is reported in many cases and may occur at diagnosis, at progression, or during relapse phase. Pericardial involvement is a rare condition that usually occurs with advanced-stage disease. We report a rare case of 76-year-old women with plasma cell-based pericardial effusion with cardiac tamponade as a form of presentation of multiple myeloma and discuss it in the light of literature. Diagnosis was established by pericardial fluid cytology. The patient received systemic chemotherapy, according to MPT protocol.

Intravascular Large B-Cell Lymphoma.

Intravascular large B-cell lymphoma (IVBCL) is a very rare and aggressive subtype of extranodal diffuse large B-cell lymphoma (DLBCL) involving the growth of lymphoma cells within blood vessels of all organ types. We present the case of a 55-year-old North-African man with no prior history of neoplastic disease presenting with weight loss and an isolated splenomegaly. Investigations led to the diagnosis of this disease. To the best of our knowledge, this is the first case recorded in Africa. Through this article, we discuss this case and outline the common presentation, paraclinical investigations, and treatment options of IVBCL.

Prevalence of Depression in Patients with Hematologic Diseases in Marrakesh, Morocco: A Single Center Experience.

Depression in patients with cancer negatively influences their physical symptoms, treatment success, coping, and quality of life and is associated with increased mortality. The reported prevalence of emotional distress in patients followed in hematology varies widely across studies. This study investigated the prevalence of depression and explored the possible associated factors in patients followed in a hematological department. Methods: in this descriptive cross-sectional study of patients followed in the hematology department of the University Hospital Mohammed VI of Marrakesh between August 2020 and December 2020, depression symptoms were assessed using Mini International Neuropsychiatric Interview test and Beck Depression Inventory. Results: 150 patients participated. Regarding their underlying pathology, 60% (90 patients) of patients were newly diagnosed with leukemia, and 8% patients were admitted for chronic anemia and 12% for lymphoma. Forty-eight percent of them had a characterized depressive episode. Four percent of these episodes were severe, 55% were moderate, and 41% were mild. Among all the factors, multivariate analysis showed that high prevalence of depression was associated with female gender and poor prognosis estimation by the patient.

Current Practice of Oral Care for Hematopoietic Stem Cell Transplant Patients: A Survey of the Eastern Mediterranean Blood and Marrow Transplantation Group.

INTRODUCTION: The oral cavity is one of the most common sites impacted by hematopoietic stem cell transplantation (HSCT) with acute complications including mucositis, bleeding, salivary gland dysfunction, infection, and taste alteration. These complications may result in significant morbidity and can negatively impact outcomes such as length of stay and overall costs. As such, oral care during HSCT for prevention and management of oral toxicities is a standard component of transplant protocols at all centers. The objective of this study was to evaluate the current oral care practices for patients during HSCT at different transplant centers within the Eastern Mediterranean region. MATERIAL AND METHODS: An internet-based survey was directed to 30 transplant centers in the Eastern Mediterranean region. The survey included five sections asking questions related to (1) transplant center demographics; (2) current oral care protocol used at the center and type of collaboration (if any) with a dental service; (3) use of standardized oral assessment tools and grading systems for mucositis; (4) consultations for management of oral complications; and (5) oral health needs at each center. Data are presented as averages and percentages. RESULTS: A total of 16 responses from 11 countries were collected and analyzed, indicating a response rate of 53%. Eight centers reported that a dentist was part of the HSCT team, with four reporting oral medicine specialists specifically being part of the team. Almost all centers (15/16; 93%) had an affiliated dental service to facilitate pre-HSCT dental clearance with an established dental clearance protocol at 14 centers (87%). Dental extraction was associated with the highest concern for bleeding and the need for platelet transfusion. With respect to infection risk, antibiotic prophylaxis was considered in the setting of low neutrophil counts with restorative dentistry and extraction. All centers provide daily reinforcement of oral hygiene regimen. The most frequently used mouth oral rinses included sodium bicarbonate (68%) and chlorhexidine gluconate (62%), in addition to ice chips for dry mouth (62%). The most frequently used mucositis assessment tools were the World Health Organization scale (7/16; 43%) and visual analogue scale for pain (6/16; 37%). Mucositis pain was managed with lidocaine solution (68.8%), magic mouth wash (68.8%) and/or systemic pain medications (75%). CONCLUSIONS: Scope and implementation of oral care protocols prior to and during HSCT varied between transplant centers. The lack of a universal protocol may contribute to gaps in oral healthcare needs and management for this group of patients. Further dissemination of and education around available oral care guidelines is warranted. CLINICAL RELEVANCE: Considering oral care during HSCT a standard component of transplant protocols, the current study highlights the common oral care practices for patients at centers within the Eastern Mediterranean region.

[Malignant hypercalcaemia revealing acute lymphoblastic leukemia: case report]. / Hypercalcémie maligne révélant une leucémie aiguë lymphoblastique : à propos d´un cas.

Malignant hypercalcaemia is a metabolic emergency. Its association with solid tumors is common, whereas it has been rarely described in patients with malignant hemopathies other than multiple myeloma and T-cell leukemias/T-cell lymphomas associated with lymphotropic virus type I (HTLV-I). We here report the case of a female patient with acute lymphoblastic leukemia revealed by malignant hypercalcaemia and pathological fracture of the humerus. Laboratory tests showed aregenerative, normocytic-normochromi anemia, hypercalcaemia (163mg/l), renal failure, creatinine (22mg/l), glomerular filtration rate 26 ml/min and low parathyroid hormone (PTH) (9.9pg/ml). X-ray of the right upper limb showed pathological fracture of humerus. Myelogram and immunophenotyping confirmed the diagnosis of LAL-B. The patient received urgent treatment for hypercalcaemia and the underlying malignancy. This study focuses attention on this atypical condition occurring during acute leukemias and on its diagnostic and therapeutic issues.

Esophageal Plasmacytoma Revealed by Persistent Hiccups: A Case Report and Literature Review.

INTRODUCTION: Solitary extramedullary plasmacytoma (SEP) is a rare neoplasm that is derived from monoclonal proliferation of plasma cells in the soft tissues or organs arising outside the bone marrow. It is present in about 3% of all plasma cell tumors and originates mainly from the upper respiratory tract and nasopharynx. Involvement of the esophagus is exceptionally seen in cases of SEP. Case Presentation. We report a novel case of a 74-year-old male patient attended with a 6-month history of hiccupping further associated with dysphasia and weight loss all caused by esophageal plasmacytoma. Histological and immunohistochemical examination of the tumor confirmed the diagnosis of plasmacytoma. Workup for the multiple myeloma came out to be negative, thus confirming the diagnosis of SEP. The patient was treated with radiotherapy alone, leading to complete remission (at 30 months of follow-up). CONCLUSION: Esophageal plasmacytoma, an exceptional presentation of extramedullary plasmacytoma, should be kept in mind while dealing with patients presenting with intractable hiccups.

Relapse of Hodgkin's disease revealed by skin involvement.

We present a case of a relapse of HL revealed by a skin involvement. A biopsy of the skin lesion showed infiltration by a mixed cellularity and Reed-Sternberg cells. The immunoreactivity was positive for CD30 and CD15. The patient was undergoing ICE protocol with good improvement after three cycles.

[Association of acute lymphoblastic leukaemia and visceral leishmaniasis]. / Association leucémie aiguë lymphoblastique et leishmaniose viscérale.

Visceral leishmaniasis (VL) can be regarded as a rare opportunistic infection in patients with acute lymphoblastic leukemia (ALL). We report the case of a 20-year-old woman treated for ALL. During maintenance treatment, the patient presents with pallor, prolonged fever and asthenia. The examination objective splenomegaly and blood counts showed pancytopenia. The bone marrow aspiration confirmed the diagnosis of VL. The patient was treated with antimoniate of meglumine with good evolution.

[Hodgkin's disease revealed by a nephrotic syndrome: a case report with literature review]. / Maladie de Hodgkin révélée par un syndrome néphrotique : à propos d'un cas avec revue de la littérature.

The association between paraneoplasic nephrotic syndrome and Hodgkin's disease is rare. We report a case of Hodgkin's lymphoma in a young female patient with nephrotic syndrome. A 40-year-old woman presented with the clinical symptoms of nephrotic syndrome, kidney biopsy revealed minimal change glomerulonephritis. A treatment with prednisone was started but the response was partial. Twenty-four months after the diagnostic of nephrotic syndrome was made, she presented prolonged fever and weight loss, the physical examination showed cervical lymphadenopathy, which lymph node biopsy revealed Hodgkin lymphoma of the nodular sclerosing type. The patient was treated for Hodgkin disease stage IVB with ABVD regimen, and was in complete remission of both Hodgkin's disease and nephrotic syndrome.

[Human immunodeficiency virus and lymphoma]. / Virus de l'immunodéficience humaine et lymphome.

Lymphomas remain a leading cause of morbidity and mortality for HIV-positive patients. The most common lymphomas include diffuse large B-cell lymphoma, Burkitt lymphoma, primary effusion lymphoma, plasmablastic lymphoma and Hodgkin lymphoma. Appropriate approach is determined by lymphoma stage, performans status, comorbidities, histological subtype, status of the HIV disease and immunosuppression. Treatment outcomes have improved due to chemotherapy modalities and effective antiretroviral therapy. This review summarizes epidemiology, pathogenesis, pathology, and current treatment landscape in HIV associated lymphoma.

[Activity report of the Bone Marrow Transplant Department of the Mohammed VI University Hospital, Marrakech, Morocco, over the period 2012- 2018]. / Bilan d'activité de l'unité de greffe de moelle osseuse du Centre Hospitalier Universitaire Mohamed VI de Marrakech, Maroc: 2012-2018.

INTRODUCTION: hematopoietic stem cell transplantation (HSCT) is part of the cellular immunotherapy arsenal. It is used in the treatment of several malignant and non-malignant hematological conditions as well as other extra-hematological diseases. HSCT has been described since 1950 and introduced in Morocco since the 2000s. GSCH is still little used in our context due to several legal, financial and organizational barriers. The purpose of this study is to report the experience of the Bone Marrow Transplant Department of the Marrakech's Mohammed VI University Hospital with hematopoietic stem cell transplantation, is one of the Hospital Departments in developing countries. METHODS: we carried out a descriptive retrospective study over a period of 6 years. RESULTS: during the study period, sixty-six HSCT were performed. Multiple myeloma was the main indication for HSCT in our case series (30 patients with autografts). In our case series, mortality rate was around 20%, relapse rate was 23% while complications rate was 38%. Despite the challenges, our results were encouraging during the long follow-up period. CONCLUSION: efforts still need to be made to improve therapy results.

[Adult non-Hodgkin bone lymphomas]. / Les lymphomes non-Hodgkiniens osseux de l'adulte.

Two forms of bone lymphomas can be distinguished: the primary bone lymphoma (PBL) and the secondary bone lymphoma (SBL). PBL is a rare disease with a good prognosis. Clinical manifestations and imaging findings are usually non-specific. Patient can present with pain, swelling of affected bone or pathologic fracture. Positron emission tomography-CT scan is a sensitive imaging modality and very useful for staging, restaging, surveillance of recurrence, and monitoring of treatment response of lymphoma. The diagnosis of PBL is often difficult and made after biopsy examination. Most patients have diffuse large B-cell lymphoma. Patients have been treated with radiotherapy, chemotherapy or combination of both. Localized disease, low IPI (International Prognostic Index) and complete remission after initial treatment were associated with a better outcome. Management of late sequelae deserves particular attention. SBL is more common than PBL; this is a disseminated lymphoma with concomitant involvement of the skeleton. We review the clinical, imaging and pathologic features of bone lymphomas; and discuss therapeutic modalities as well as prognosis of these lymphomas in the era of immunochemotherapy.

Neutropenic Enterocolitis Disclosing an Underlying Cyclic Neutropenia.

Neutropenic enterocolitis is a syndrome characterized by fever and abdominal pain in a neutropenic patient. It is often reported in children treated for leukemia and rarely reported in patients with other diseases. Herein, we report the case of a 9-year-old patient with a medical history of recurrent fever and mouth ulcers since the age of 4, who presented with neutropenic enterocolitis complicated with intestinal perforation which all leaded to disclose cyclic neutropenia. The patient was successfully treated by aggressive supportive care combined with surgical intervention. Neutropenic enterocolitis with possible complications should be considered and promptly managed in every neutropenic patient and may reveal a rare cause of neutropenia as cyclic neutropenia.

[Hepatocellular failure revealing T-cell large granular lymphocyte leukemia: Morocco case study and literature review]. / Un syndrome d'insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d'un cas marocain et revue de la littérature.

In the last classification of the World Health Organization (WHO) 2016, T-cell large granular lymphocyte (LGL) leukemia is a type of mature natural killer T (NKT) cell lymphatic hematologic disease. T-LGL leukemia is characterized by CD3+ T-cell phenotype and TCR gene rearrangement (T lymphocyte receptor). It is a rare disease, mainly affecting people living in the Western world. We report the case of a 60-year old Moroccan patient presenting with 1-month history of generalized jaundice with diffuse petechiae and impaired general condition. Initial clinical examination showed ascites of average abundance and hepatosplenomegaly; paraclinical examination objectified hepatocellular failure with neutropenia and thrombocytopenia. The diagnosis of T-LGL was retained based on cytological and immunophenotypic study of bone marrow blood as well as on the detection of TCR rearrangement using molecular test. The patient received cyclophosphamide associated with symptomatic treatment. The patient died within three months of the diagnosis due to septic shock with cachexia. T-LGL often has an indolent behavior and initial clinical status at diagnosis is generally moderate, with progressive onset but that was not so for our patient. Hepatocellular failure exceptionally results in T-cell large granular lymphocyte (LGL) leukemia.

[Severe acquired marrow aplasia and seronegative autoimmune hepatitis: a rare and serious association]. / L'aplasie médullaire acquise sévère et l'hépatite auto-immune séronégative: une association rare et grave.

Post-hepatitis marrow aplasias are severe aplasias occurring in the six months following at least one episode of early onset hepatitis, usually seronegative for hepatitis viruses known. We report the case of a 28-year old patient with autoimmune hepatitis revealed by jaundice associated with hepatomegaly and hepatic cytolysis and confirmed by liver biopsy; four months later it was complicated by pancytopenia associated with severe marrow aplasia.