A rare association of a high grade glioblastoma, cerebral abscess and acute lymphoblastic leukemia in a child with Noonan syndrome.

Noonan syndrome is a genetic disorder frequently caused by PTPN11 mutations. Patients with Noonan syndrome are characterized by facial dysmorphism, short stature and congenital heart defects and they have a reported predisposition to malignancies such as leukemia, and solid and central nervous system tumors. Here, we report a case of a 14-year-old boy with Noonan syndrome treated for T-cell acute lymphoblastic leukemia who presented with 2 concomitant abnormalities: cerebral abscess and high grade glioblastoma. This exceptional association exhibits to a poorer prognosis and may sometimes delay the diagnosis and therefore the therapeutic intervention.

Evaluation of glycopeptide prescription in high-risk febrile neutropenia: A monocentric study of North Africa.

INTRODUCTION: High-risk febrile neutropenia (FN) is one of the main causes of morbidity and mortality in onco-hematology. The initiation of empirical antibiotic therapy is an emergency that can change the prognosis of some patients. Given the emergence of increasingly resistant Gram-positive bacteremia, glycopeptides, as an empirical treatment, have an important place in the management of high-risk FN. The aim of this study is to evaluate the appropriateness of glycopeptide prescription in high-risk FN patients. METHODS: This study was conducted in the Hematology Department of Fattouma Bourguiba University Hospital of Monastir, Tunisia. Patients with high-risk FN were enrolled during the period between January 1 and December 31, 2020. RESULTS: Of the 29 patients included in this study, 88 FN episodes were noted of which 39 episodes treated with glycopeptides were evaluated. Twenty-four febrile episodes were empirically treated with glycopeptides (27.3%) of which 17 prescriptions (70.8%) were appropriate according to the European Conference on Infection in Leukemia and the Infectious Diseases Society of America recommendations. A therapeutic escalation using glycopeptides was noted in 17% of cases and appropriately opted in 6 FN episodes (40%). CONCLUSION: Prescriptions of glycopeptides were appropriate according to the international recommendations in 71% of the empirical prescriptions and in 40% of the therapeutic escalation using glycopeptides. In high-risk FN episodes, glycopeptides prescriptions should be rationalized and limited to the indications detailed in the international guidelines to control the emergence of multidrug-resistant bacteria.

Chronic-phase chronic myeloid leukemia: Incidence of BCR/ABL transcript and its correlation with presenting features, response to treatment, and survival.

Introduction: Chronic myeloid leukemia (CML) is characterized by Philadelphia chromosome resulting in the fusion between the BCR gene, located on chromosome 22, and the ABL gene on chromosome 9. The prognostic significance of BCR-ABL transcript variants in CML is controversial. The aim of the current study was to evaluate the clinico-hematological presentation and evolution of the disease, response to treatment and survival according to transcript type in chronic phase CML patients. Results: The median age of our population was 50 years with a slight female predominance (sex-ratio 0.78). Sixty percent had the b3a2 transcript and 34% had the b2a2 type. Patients with the co-expression of these two transcripts (4.5%) and those with e19a2 were excluded from the analysis. Patients with b3a2 subtype were associated significantly with thrombocytosis (p = 0.006) and higher Sokal score (p = 0.038) compared to those with b2a2 transcript. The two isolated transcripts were not significantly associated with gender, age group, blast cell percentage or the identified ranges of spleen size. Complete cytogenetic response at 12 months for b3a2 patients and b2a2 patients was 78.6% and 21.4% respectively. This difference was statistically significant (p = 0.001, HR = 9.5, 95% CI 6.5-13.7). Patients with b3a2 transcript had a higher rate of optimal molecular response at 3 months (p = 0.04, HR = 4.2, 95% CI 1-17.3) and major molecular response at 12 months (p = 0.004, HR = 4.9, 95%CI 1.5-15.1). At the date of last follow-up, most patients achieving deep molecular response (MR4 or deeper) belonged to b3a2 group (79%) (p = 0.003, HR = 5.2, 95% CI 1.6-16.4). We did not find a significant difference in OS and EFS between the two groups. Conclusion: Our study concluded that b2a2 transcript is a prognostic factor in cytogenetic and molecular response but further studies are needed to complete this aspect.

Autoimmune hemolytic anemia complicating a chronic neutrophilic leukemia: A case report of a rare association.

Key Clinical Message: Chronic neutrophilic leukemia is a rare disease with a poor prognosis. Its diagnosis is challenging in the lack of genetic tools. It can infrequently be associated with autoimmune hemolytic anemia. Abstract: Chronic neutrophilic leukemia is a rare disease with poor prognosis, characterized by a sustained mature neutrophilic leukocytosis in the absence of monocytosis or basophilia with few or no circulating immature granulocytes, hepatosplenomegaly, and granulocytic hyperplasia of the bone marrow. In addition, no molecular markers for other myeloproliferative neoplasms are detected. The 2016 WHO classification included the presence of the CSF3R mutation as a key diagnostic criterion for this disease. Although anemia may be present at diagnosis, hemolytic one rarely complicates myeloproliferative neoplasms. Treatment is largely based on cytoreductive agents, but bone marrow allograft remains the only curative option. We report the case of a patient with chronic neutrophilic leukemia associated with autoimmune hemolytic anemia. We describe the epidemiological, clinical, prognostic, and therapeutic features of this disease in addition to the difficulties of its diagnosis and management in Tunisia.

High-dose methotrexate-related pneumonitis in a child with acute lymphoblastic leukemia.

INTRODUCTION: Methotrexate is administered through different routes to treat childhood acute lymphoblastic leukemia. Toxicities of low, intermediate, or high doses of methotrexate have been described in the literature. Methotrexate-induced or related pneumonitis is a rare complication that leads, in most cases, to discontinuing this drug. CASE REPORT: We report a case of a 17-year-old female patient with newly diagnosed B-acute lymphoblastic leukemia who received a high-dose methotrexate on an induction course. Eight days after methotrexate infusion, she developed fever, dyspnea, hypoxemia, and dry cough. Chest computed tomography showed mosaic attenuation of lung parenchyma and bilateral interstitial infiltrate in favor of hypersensitivity pneumonitis with no evidence of bacterial or fungal infections. MANAGEMENT AND OUTCOME: Methotrexate-related pneumonitis was diagnosed and corticosteroids were prescribed. Improvement of the symptomatology was noted within four days. Given the importance of methotrexate in the treatment of acute lymphoblastic leukemia, intrathecal methotrexate was administered without incident and high-dose methotrexate was re-introduced successfully two and a half months after the pneumonitis episode while using corticosteroids. According to Naranjo's algorithm, the reaction to the drug was found to be probable with a score of 6. DISCUSSION: Methotrexate is the backbone of acute lymphoblastic leukemia treatment, but numerous adverse reactions have been published of which methotrexate pneumonitis can be fatal in some cases. In this case, we concluded that this drug can be successfully reintroduced after methotrexate-related pneumonitis.

The diagnostic dilemma of idiopathic intracranial hypertension in a child with acute lymphoblastic leukemia: COVID-19 or cytosine arabinoside?

BACKGROUND: Idiopathic intracranial hypertension is a rare neurological condition among children. Its manifestations vary from mild headaches to loss of vision. Although rare, COVID-19 infection and high dose cytosine arabinoside have been reported as risk factors for this neurological disorder. In patients with acute leukemia, idiopathic intracranial hypertension diagnosis is simple, but finding its etiology can be difficult. CASE PRESENTATION: We report a case of a 9-year-old boy with an ongoing treatment for T-acute lymphoblastic leukemia presenting with persistent headaches and diplopia. A diagnosis of idiopathic intracranial hypertension was retained based on clinical, imaging and laboratory findings. Due to its rarity, we describe its clinical and therapeutic features and highlight the challenging etiological dilemma between COVID-19 infection and high dose cytosine arabinoside administration. CONCLUSION: Persistent headache in a pediatric patient with leukemia can be due to many neurological disorders other than leukemic relapse. Given the improvement of the neurological symptoms after the SARS-CoV-2 PCR negativization and the successful re-introduction of high dose cytosine Arabinoside, the diagnosis of idiopathic intracranial hypertension associated with Covid-19 infection was withheld.