Cytopenia
View article: PO:22:033 | Unraveling VEXAS syndrome: when skin manifestations and monoclonal gammopathy precede hematological myeloid alterations
PO:22:033 | Unraveling VEXAS syndrome: when skin manifestations and monoclonal gammopathy precede hematological myeloid alterations Open
Background. VEXAS (Vacuoles, Enzyme E1, X-linked, Autoinflammatory, Somatic) syndrome is a rare adult disease caused by somatic mutations in the UBA1 gene within hematopoietic stem cells (1). These mutations compromise the ubiquitin-activa…
View article: Severe cutaneous adverse reactions to anti-osteoporosis drugs: a real-world pharmacovigilance study using the FDA Adverse Event Reporting System database and a review of published cases
Severe cutaneous adverse reactions to anti-osteoporosis drugs: a real-world pharmacovigilance study using the FDA Adverse Event Reporting System database and a review of published cases Open
Objective This study analyzed severe cutaneous adverse reactions (SCARs) linked to anti-osteoporosis drugs using FDA Adverse Event Reporting System (FAERS) data and characterized implicated drugs and clinical features through a literature …
View article: Supplementary Figure 2 from Therapy-related Myeloid Neoplasms Following PARP Inhibitors: Real-life Experience
Supplementary Figure 2 from Therapy-related Myeloid Neoplasms Following PARP Inhibitors: Real-life Experience Open
Blood smear and bone marrow aspiration of patient referred for cytopenia post PARPi.
View article: Hemophagocytic Lymphohistiocytosis in a Pediatric Lung Transplant Recipient
Hemophagocytic Lymphohistiocytosis in a Pediatric Lung Transplant Recipient Open
Background Hemophagocytic lymphohistiocytosis (HLH) is a rare complication of solid organ transplantation and is a syndrome of systemic hyperinflammation secondary to dysregulation of the inflammatory response, primarily involving lymphocy…
View article: Supplementary Figure 3 from Therapy-related Myeloid Neoplasms Following PARP Inhibitors: Real-life Experience
Supplementary Figure 3 from Therapy-related Myeloid Neoplasms Following PARP Inhibitors: Real-life Experience Open
Waffle chart (%) of patients addressed for cytopenia exploration after OC exposed to a PARPi treatment from cytopenia diagnosis (A) and cytopenia diagnosis based on NGS results (B). Dot plots (C) showing the median WBC, hemoglobin, and pla…
View article: Integrated Multimodal Diagnostic Validation in Hematological Disorders: First Prospective Study from a Conflict-Affected, Resource-Limited Setting in Yemen
Integrated Multimodal Diagnostic Validation in Hematological Disorders: First Prospective Study from a Conflict-Affected, Resource-Limited Setting in Yemen Open
Background and objectives: Hematological disorders pose significant diagnostic challenges, particularly in conflict-affected, resource-limited regions like Yemen, leading to delays, misdiagnoses, and suboptimal patient care. This study aim…
View article: Novel Therapeutic Approaches in Pediatric Acute Lymphoblastic Leukemia
Novel Therapeutic Approaches in Pediatric Acute Lymphoblastic Leukemia Open
Acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy, characterized by the clonal proliferation of immature lymphoid precursors. The distinction between B-cell ALL (B-ALL) and T-cell ALL (T-ALL) is fundamental, as eac…
View article: Hematopoietic cell transplantation for pediatric myelodysplastic syndromes
Hematopoietic cell transplantation for pediatric myelodysplastic syndromes Open
Myelodysplastic syndromes (MDS) are uncommon in children and present distinct features compared to adults. Allogeneic hematopoietic cell transplantation (HCT) is considered the standard curative treatment for many children with MDS, includ…
View article: Piperacillin-tazobactam-induced hemophagocytic lymphohistiocytosis in a patient with community-acquired pneumonia: A case report and literature review on diagnostic challenges of elevated procalcitonin
Piperacillin-tazobactam-induced hemophagocytic lymphohistiocytosis in a patient with community-acquired pneumonia: A case report and literature review on diagnostic challenges of elevated procalcitonin Open
Rationale: piperacillin-tazobactam, a widely used broad-spectrum antibiotic, carries a risk of severe adverse reactions, including rare but life-threatening hemophagocytic lymphohistiocytosis (HLH). Elevated procalcitonin (PCT), typically …
View article: Lymphoma-associated hemophagocytic lymphohistiocytosis
Lymphoma-associated hemophagocytic lymphohistiocytosis Open
Hemophagocytic lymphohistiocytosis is a severe, rare condition characterized by excessive immune activation, leading to significant morbidity and mortality. Lymphoma is the most common trigger for malignancy-related hemophagocytic lymphohi…
View article: Acquired severe aplastic anemia
Acquired severe aplastic anemia Open
Acquired severe aplastic anemia is a rare, life-threatening condition characterized by pancytopenia and hypocellularity of the bone marrow. It shows a bimodal age distribution, with approximately 70–80% of cases classified as idiopathic. R…
View article: Diagnosis and Management of Visceral Leishmaniasis in Children: A French Retrospective Study
Diagnosis and Management of Visceral Leishmaniasis in Children: A French Retrospective Study Open
Introduction Few studies have investigated the diagnosis and management of pediatric visceral leishmaniasis (VL) in high-income countries. Methods All children hospitalized for VL in university hospitals across mainland France between Janu…
View article: Utility and Safety of Romiplostim in Pediatric Allogeneic Stem Cell Transplantation
Utility and Safety of Romiplostim in Pediatric Allogeneic Stem Cell Transplantation Open
Background The use of romiplostim, a thrombopoietin agonist, has increased in the last decade for the treatment of immune mediated thrombocytopenia and severe aplastic anemia. Its utility has been explored in the management of delayed plat…
View article: LASSA FEVER COMPLICATED BY ACUTE RESPIRATORY DISTRESS SYNDROME IN A HEALTHCARE WORKER: LESSON FROM A SUCCESSFUL MANAGEMENT EXPERIENCE AT FMC OWO
LASSA FEVER COMPLICATED BY ACUTE RESPIRATORY DISTRESS SYNDROME IN A HEALTHCARE WORKER: LESSON FROM A SUCCESSFUL MANAGEMENT EXPERIENCE AT FMC OWO Open
Lassa fever, a viral hemorrhagic disease endemic to West Africa, remains a major public health concern, particularly when complicated by acute respiratory distress syndrome (ARDS). Pulmonary involvement is relatively uncommon, occurring in…
View article: 51 A Comprehensive Analysis of Clinical, Pathological, and Genetic Profiles of Indian Cohort of Patients with VEXAS Syndrome
51 A Comprehensive Analysis of Clinical, Pathological, and Genetic Profiles of Indian Cohort of Patients with VEXAS Syndrome Open
The VEXAS syndrome is a recently recognized, acquired monogenic adult onset haemato-inflammatory syndrome defined by somatic mutations within the UBA1 gene. It is an acronym that stands for vacuoles, E1 enzyme, X-linked inheritance, autoin…
View article: 65 Hemophagocytic Lymphohistiocytosis Complicated by Pulmonary Mucormycosis in an Immunocompromised Acute Myeloid Leukemia Patient: A Case Report
65 Hemophagocytic Lymphohistiocytosis Complicated by Pulmonary Mucormycosis in an Immunocompromised Acute Myeloid Leukemia Patient: A Case Report Open
Introduction/Objective Hemophagocytic lymphohistiocytosis (HLH) is a rare, hyper-inflammatory syndrome first described by Scott and Robb-Smith in 1939. It is characterized by dysregulation in natural killer cells and cytotoxic T-cell funct…
View article: 71 Immune Thrombocytopenia and Thrombotic Thrombocytopenic Purpura: A Case of Overlapping Diagnoses in a Single Patient with Literature Review
71 Immune Thrombocytopenia and Thrombotic Thrombocytopenic Purpura: A Case of Overlapping Diagnoses in a Single Patient with Literature Review Open
Introduction/Objective Immune thrombocytopenia (ITP) and thrombotic thrombocytopenic purpura (TTP) are distinct disorders involving autoimmune platelet destruction and ADAMTS13 deficiency, respectively. Overlap of ITP and TTP diagnoses is …
View article: Leukopenia of Undetermined Significance in a Person with Long-term Exposure to Ionizing Radiation: a Perspective on Clonal Hematopoiesis (Clinical Case)
Leukopenia of Undetermined Significance in a Person with Long-term Exposure to Ionizing Radiation: a Perspective on Clonal Hematopoiesis (Clinical Case) Open
Relevance: The phenomenon of clonal hematopoiesis (CH) is usually considered as a biological condition that precedes the development of a number of diseases and pathological processes, primarily haematological malignancies (HM). The concep…
View article: CLINICAL IMPACT OF CHEMOTHERAPY ON BLOOD CELL PARAMETERS IN KASHMIR CANCER PATIENTS
CLINICAL IMPACT OF CHEMOTHERAPY ON BLOOD CELL PARAMETERS IN KASHMIR CANCER PATIENTS Open
Cancer treatments continue to be the backbone of cancer care, but the harmful side effect of chemotherapy is systemic harm to the bone marrow and can cause myelosuppression that can result in anemia, neutropenia and thrombocytopenia. Myelo…
View article: CLINICAL IMPACT OF CHEMOTHERAPY ON BLOOD CELL PARAMETERS IN KASHMIR CANCER PATIENTS
CLINICAL IMPACT OF CHEMOTHERAPY ON BLOOD CELL PARAMETERS IN KASHMIR CANCER PATIENTS Open
Cancer treatments continue to be the backbone of cancer care, but the harmful side effect of chemotherapy is systemic harm to the bone marrow and can cause myelosuppression that can result in anemia, neutropenia and thrombocytopenia.…
View article: 258 AML with t(8;21) and B-lineage Marker Expression: A Diagnostic Crossroad
258 AML with t(8;21) and B-lineage Marker Expression: A Diagnostic Crossroad Open
Introduction/Objective Acute myeloid leukemia (AML) with t(8;21)(q22;q22) is a distinct subtype characterized by RUNX1-RUNX1T1 fusion. Although AML typically expresses myeloid markers, aberrant expression of B-lineage markers can occur, co…
View article: Clinical Characteristics and Reported Cases of VEXAS Syndrome in the United States: A Scoping Review
Clinical Characteristics and Reported Cases of VEXAS Syndrome in the United States: A Scoping Review Open
Background VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset disorder caused by somatic mutations in the UBA1 gene. It primarily affects older men and is characterized by systemic inflammation and …
View article: Recurrent Immune Thrombocytopenic Purpura in a Pediatric Patient: A Case of Viral-Triggered Autoimmunity and Therapeutic Challenges
Recurrent Immune Thrombocytopenic Purpura in a Pediatric Patient: A Case of Viral-Triggered Autoimmunity and Therapeutic Challenges Open
We report a case of refractory idiopathic thrombocytopenic purpura (ITP) in a patient with recurrent relapses despite conventional therapy, including corticosteroid tapering (deflazacort), adjuvant pimotimod (for recurrent infections and a…
View article: P145 | Persistent fever of unknown origin and bilinear cytopenia of new onset in healthy woman
P145 | Persistent fever of unknown origin and bilinear cytopenia of new onset in healthy woman Open
Premises: A 65-year-old woman was admitted for persisting fever, anaemia (Hb 9.7 g/dL) and thrombocytopenia (PLTs 52.000/mmc).Discussion: Upon physical examination, a single leg skin ulcer and fever were found. Laboratory results showed le…
View article: CO08 | Two cases of post-traumatic hemophagocytic lymphohistiocytosis: a potential cause of fever and cytopenia after trauma
CO08 | Two cases of post-traumatic hemophagocytic lymphohistiocytosis: a potential cause of fever and cytopenia after trauma Open
Background: Post-traumatic hemophagocytic lymphohistiocytosis (PT-HLH) is a rare and potentially life-threatening condition characterized by uncontrolled activation of the immune system. The diagnosis is often made in the setting of exclus…
View article: Case Report: FAS spontaneous mutation in a familial hemophagocytic lymphohistiocytosis patient with a complex heterozygous mutation in PRF1
Case Report: FAS spontaneous mutation in a familial hemophagocytic lymphohistiocytosis patient with a complex heterozygous mutation in PRF1 Open
Familial hemophagocytic lymphohistiocytosis type 2 (FHL2), caused by perforin 1 (PRF 1), is a rare and fatal autosomal recessive disorder characterized by a hyperinflammatory syndrome and the accumulation of activated T lymphocytes and his…
View article: Case Report: Eltrombopag in mosaic and gene therapy-treated patients with Fanconi anemia
Case Report: Eltrombopag in mosaic and gene therapy-treated patients with Fanconi anemia Open
Fanconi anemia (FA) constitutes the most common of the inherited bone marrow failure syndromes, a group of rare heterogeneous disorders characterized by cytopenia, predisposition to hematologic and solid malignancies and diverse clinical f…
View article: Pediatric Hemophagocytic Lymphohistiocytosis Presenting as a Refractory Febrile Illness: A CASE REPORT
Pediatric Hemophagocytic Lymphohistiocytosis Presenting as a Refractory Febrile Illness: A CASE REPORT Open
BackgroundHemophagocytic Lymphohistiocytosis (HLH) is a life-threatening, exceedingly rare hyperinflammatory syndrome that typically presents with nonspecific symptoms such as fever and cytopenias. An early diagnosis of HLH is a significan…
View article: A pilot transcriptomic study of a novel multitargeted BRT regimen for anti–MDA5 antibody-positive dermatomyositis: improving survival over conventional therapy
A pilot transcriptomic study of a novel multitargeted BRT regimen for anti–MDA5 antibody-positive dermatomyositis: improving survival over conventional therapy Open
Background Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (MDA5-DM) is associated with severe outcomes, primarily due to rapidly progressive interstitial lung disease (RP-ILD), which is often refractory t…
View article: Retrospective analysis of ruxolitinib as induction treatment in pediatric hemophagocytic lymphohistiocytosis
Retrospective analysis of ruxolitinib as induction treatment in pediatric hemophagocytic lymphohistiocytosis Open
Purpose: To retrospectively evaluate the efficacy and safety of ruxolitinib, with or without intensive chemotherapy, in pediatric patients with hemophagocytic syndrome.Methods: Pediatric patients with newly diagnosed hemophag…