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The SARS-CoV-2 pandemic still represents a threat for immunosuppressed and hematological malignancy (HM) bearing patients, causing increased morbidity and mortality. Given the low anti-SARSCoV-2 IgG titers post-vaccination, the COVID-19 threat prompted the prophylactic use of engineered anti-SARS-CoV-2 monoclonal antibodies. In addition, potential clinical significance of T cell responses has been overlooked during the first waves of the pandemic, calling for additional in-depth studies. We reported that the polarity and the repertoire of T cell immune responses govern the susceptibility to SARS-CoV-2 infection in health care workers and solid cancer patients. Here, we longitudinally analyzed humoral and cellular immune responses at each BNT162b2 mRNA vaccine injection in 47 HM patients under therapy. Only one-third of HM, mostly multiple myeloma (MM) bearing patients, could mount S1-RBD-specific IgG responses following BNT162b2 mRNA vaccines. This vaccine elicited a S1-RBD-specific Th1 immune response in about 20% patients, mostly in MM and Hodgkin lymphoma, while exacerbating Th2 responses in the 10% cases that presented this recognition pattern at baseline (mostly rituximab-treated patients). Performing a third booster barely improved the percentage of patients developing an S1-RBD-specific Th1 immunity and failed to seroconvert additional HM patients. Finally, 16 patients were infected with SARS-CoV-2, of whom 6 developed a severe infection. Only S1-RBD-specific Th1 responses were associated with protection against SARS-CoV2 infection, while Th2 responses or anti-S1-RBD IgG titers failed to correlate with protection. These findings herald the paramount relevance of vaccine-induced Th1 immune responses in hematological malignancies. [ABSTRACT FROM AUTHOR]

Germline mutations in adult AML patients are uncommon and often associated with organ dysfunction (i.e. I GATA2 i deficiency [[6]]) or a preexisting hematologic disorders (i.e. thrombocytopenia in I RUNX1 i mutated patients [[7]]), but could also found in de "novo" patients (i.e. I CEBPA i [[8]] and I DDX41 i [[9]]). Three patients (50%) had extramedullary localization (nodal involvement, hepatosplenomegaly and skin lesion in 3, 2, and 1 patients respectively), compared to 11/72 (15%) for other AML patients. As we treated patients from this geographic area with familial history of AML but no personal history of MPN, we wondered if CNVdup14 could arise in " I de novo i AML" patients. Genetic predisposition to acute myeloid leukemia (AML) concerns a minority of adult patients. [Extracted from the article]

The article focuses on determination of retroperitoneal fibrosis (RPF) as extramedullary hematopoiesis of a chronic myelomonocytic leukemia (CMML). It mentions that CMML-associated RPF that remained unresponsive to steroids but dramatically improved upon treatment with the demethylating drug azacytidine. It states that the occurrence of an autoimmune or inflammatory disorder, mostly a vasculitis, an arthritis or a relapsing polychondritis is a well-known feature in CMML.

A 37-year-old man presented a slight debility. The hemogram showed a phenotype of β-thalassemia minor: Hb (13.1 g/dL), mean corpuscular volume (MCV) (62 fL) with low mean corpuscular hemoglobin (MCH) (20.8 pg), associated with a high level of Hb A2 of 5.3%. The serum ferritin level was 1,072 ng/mL. The sequencing of the mutated fragment revealed a duplication of four bases of codons 7/8 involving a shift in the open reading frame starting from codon 9 with a TGA stop codon at codon 23: codons 7/8/9 (+AGAA); GAG.AAG.TCT(Gly-Lys-Ser)>GAG.AA AGAAG. The human hemoglobin (Hb) instability tests were negative. The patient did not present the high iron Fe (HFE) mutation (C282Y, H63D). The same mutation was found in five other unrelated families (representing a total of 23 patients). All of their ancestors came from the north of France. This mutation has not been described before and could have its origins in the native populations of Northern France. [ABSTRACT FROM AUTHOR]