Leucémie à plasmocytes : A propos de 5 observations cliniques: Plasma cell leukemia: report of 5 cases report

Plasma cell leukemia is a malignant plasma cell proliferation in bone marrow and peripheral blood. It is the most agressive form of plasma cell malignancies. It occurs in two forms: primary and secondary plasma cell leukemia. Plasma cell leukemia is defined by the presence of more than 2Giga/l peripheral blood plasma cells or plasmocytosis more than 20% of leukocyte count. In this context, we report 5 observations of plasma cell leukemia (1 case of primary plasma cell leukemia and 4 cases of secondary plasma cell leukemia) encountered during 9 years in Farhat Hached Hospital in Sousse-Tunisia. Through these observations, we describe clinical, paraclinical, prognostic and therapeutic characteristics of this pathology. La leucémie à plasmocytes est une prolifération maligne de cellules plasmocytaires dans la moelle osseuse et le sang périphérique. Elle représente la forme la plus agressive des néoplasies plasmocytaires. Elle se présente sous deux formes : une forme primitive et une forme secondaire compliquant un myélome multiple. Elle est définie par une plasmocytose sanguine supérieure à 2 Giga/l ou un taux de plasmocytes supérieur à 20% des leucocytes. Dans ce contexte, nous rapportons 5 observations de leucémie à plasmocytes (1 cas de leucémie à plasmocytes primitive et 4 cas de leucémie à plasmocytes secondaire) survenus en 9 ans à l’hôpital Farhat Hached de Sousse (Tunisie). A travers ces observations, nous décrivons les caractéristiques cliniques, paracliniques, pronostiques et thérapeutiques de cette pathologie. &nbsp

Acute Pancreatitis in Children with Acute Lymphoblastic Leukemia Using L-Asparaginase: A Review of the Literature

L-asparaginase (L-Aspa) is utilized as a part of the therapy in children with acute lymphoblastic leukemia (ALL), achieving remission in 83–95% of the younger patients. Hypersensitivity reactions, as well as liver and pancreatic cytotoxicity, are severe documented side effects. L-Aspa-induced acute pancreatitis (AP) has been observed in 2.5–16% of treated patients. Patients with mild pancreatitis may be retreated with L-Aspa if they have no clinical symptoms within 48 hours, amylase and lipase levels are less than three times the normal’s upper limit, and there is no evidence of pseudocysts or necrosis on imaging. It is crucial to monitor patients under L-Aspa therapy, through careful observation of clinical signs and laboratory follow-up, as well as a continuous checkup for associated medications

Oral Lichen Planus and Polycythemia: Possible Association

Oral lichen planus is a chronic inflammatory disease of established immune-mediated pathogenesis that affects the oral mucosa. Polycythemia is a nonaggressive myeloproliferative disorder, characterized by an increase in red blood cell mass, often with uncontrolled production of granulocytes and platelets. Their association was rarely mentioned in the scientific literature. The aim of this paper was to report their occurrence in a 52-year-old male patient. Although a casual connection cannot be excluded, both diseases share many similarities in the immune dysfunctions involved in their pathogenesis and their clinical features. Such a hypothesis remains to be demonstrated by further studies. The presence of oral lesions should alert the clinicians in the process of identifying and early diagnosing these diseases. Thus, complications can be prevented and treatment can be started at an early stage, avoiding further damage

Meningococcemia complicated by myocarditis in a 16-year-old young man: a case report

Fulminant meningococcemia is a relatively rare life-threatening disease caused by Neisseria meningitidis. The clinical presentation is varied, but, when associated with myocarditis, it carries a particularly poor prognosis. We report a case of a patient with fulminant meningococcemia who subsequently developed severe myocardial dysfunction and successfully recovered within a period of 7 days of hospitalization. A 15-year-old girl presented with headache, fever, body ache for 1 day and few ecchymotic rash over her body for 3 hours. Blood cultures confirmed infection with N. meningitidis. After 2 days in the hospital, the patient developed dyspnea, elevated jugular venous pressure and shock. The patient was managed with intravenous ceftriaxone, furosemide and norepinephrine. Over the next 4 days the patient rapidly improved. Meningococcemia complicated by myocarditis has an extremely poor prognosis with high mortality. Our case suggests that recovery from a severe myocardial dysfunction can occur rapidly within a few days. Prompt recognition and management in this case might have contributed to the patient's rapid recovery from myocarditis

IKZF1 genetic variants rs4132601 and rs11978267 and acute lymphoblastic leukemia risk in Tunisian children: a casecontrol study

Background Associations between IKZF1 gene variants and Acute Lymphoblastic Leukemia (ALL) was recently reported. We examined whether the common IKZF1 polymorphisms rs4132601 T/G and rs111978267 A/G are associated with ALL among a Tunisian pediatric cohort. Methods This case-control study involved 170 patients with ALL and 150 control subjects. SNP genotyping was performed by TaqMan® SNP Genotyping Assay

Success of anti-CD20 monoclonal antibody treatment for severe autoimmune hemolytic anemia caused by warm-reactive immunoglobulin A, immunoglobulin G, and immunoglobulin M autoantibodies in a child: a case report

Abstract Background Autoimmune hemolytic anemia is rare in children. First-line therapies for this disease consist of corticosteroids and intravenously administered immunoglobulin that are effective in most patients. However, a small proportion of cases (5 to 10%) is refractory to these therapies and may represent a medical emergency, especially when hemolysis is due to warm immunoglobulin M. Recently, reports of the use of rituximab in adult autoimmune diseases have shown promising results. In children, there are few studies on the use of rituximab in the treatment for autoimmune hemolytic anemia, especially on its long-term efficacy and adverse effects. Case presentation Here, we report the case of a 10-year-old Tunisian girl with refractory acute autoimmune hemolytic anemia caused by warm-reactive immunoglobulin A, immunoglobulin G, immunoglobulin M, and C3d autoantibodies. First-line treatments using corticosteroids and intravenously administered immunoglobulin were ineffective in controlling her severe disease. On the other hand, she was successfully treated with rituximab. In fact, her hemolytic anemia improved rapidly and no adverse effects were observed. Conclusions The case that we report in this paper shows that rituximab could be an alternative therapeutic option in severe acute autoimmune hemolytic anemia with profound hemolysis refractory to conventional treatment. Moreover, it may preclude the use of plasmapheresis in such an urgent situation with a sustained remission

Acquired hemophilia A following COVID-19 vaccine: a case report

Abstract Background In the literature, reported cases of Acquired hemophilia A (AHA) induced by COVID-19 vaccination occurred after Adenoviral Vector Deoxyribonucleic Acid (DNA)- and SARS-CoV-2 Messenger Ribonucleic acid (mRNA)-Based vaccines. Here, and to the best of our knowledge, we report the first case of AHA occurring after an inactivated Sinovac-coronavac COVID-19 vaccine. Case presentation A 69-year-old Tunisian male patient consulted for severe left leg pain limiting physical mobility due to a 5*6 cm large ecchymosis located at the left inner thigh, having spontaneously appeared 5 days prior consultation and without notion of trauma. The patient had no known personal medical history. He had received the second dose of CoronaVac-SinoVac vaccine 30 days prior to consultation. Further physical examination revealed the presence of two other ecchymoses: one at the inner face of the right forearm, starting at the wrist reaching the elbow and the other at the left flank of the abdomen. Diagnosis of AHA was based on clinical presentation and confirmed with prolonged a PTT, Factor VIII deficiency and the presence of an FVIII inhibitor. The patient was successfully treated with corticosteroids and low dose Rituximab. Conclusion Clinicians should consider AHA in front of prolonged aPTT with or without spontaneous bleedings even after inactivated virus COVID-19