Orbital involvement is one of the extramedullary manifestations in acute leukemia. It is common in acute myeloid leukemia, but rare in acute lymphoblastic leukemia (ALL). We described a 3-year-old girl who presented with progressive proptosis of the right eye and was later diagnosed with precursor B-cell ALL. Initial blood count showed Hb 6.9 g/dL, WBC 42,000/mm3, lymphoblast 50%, and platelet count 185,000/mm3. Bone marrow aspiration revealed 90% lymphoblasts with positivity for CD10, CD19, CD20, CD22, and HLA-DR markers. Computed tomography (CT) scan of the brain and orbit revealed a homogeneous enhancing mass involving the right orbit with intracranial extension. The cytogenetic study showed 46,XX chromosomes. After 4 weeks of induction chemotherapy for very high-risk ALL, although the bone marrow was in remission, the proptosis was partially resolved. CT scan confirmed a decrease in size of the right orbital mass and degree of intracranial extension. Unfortunately, the patient abandoned the treatment after the induction chemotherapy. The actual incidence of orbital involvement in ALL is unknown. Previous case reports describe diverse manifestations of orbital involvement in ALL. The involvement may be unilateral or bilateral, may occur at first diagnosis or at relapse, and may be seen in isolation or with other systemic symptoms. There is no standard treatment protocol. Chemotherapy with or without radiotherapy is generally suggested. The role of upfront hematopoietic stem cell transplantation remains inconclusive. The previously reported prognosis of ALL with orbital involvement is poor.
Hemophilia is an inherited bleeding disorder caused by deficiency of a specific coagulation factor. Factor VIII deficiency is responsible for hemophilia A while factor IX deficiency is responsible for hemophilia B. As per the 2020 annual global survey by the World Federation of Hemophilia, only 1828 Thai hemophiliacs have been registered to the national healthcare system. The reason for the low number is the underdiagnosis which is a major concern in the real-world practice among Asian countries. In Thailand, most hemophiliacs are diagnosed by general practitioners, pediatricians or internists at rural hospitals and are referred to hemophilia specialists at the Hemophilia Treatment Centers (HTCs). Despite the challenges pertaining to infrastructure and cost of treatment, Thailand has progressed substantially in providing the required hemophilia care, as evidenced by an evolution in acquiring and sharing knowledge as well as collaborative efforts among multiple stakeholders over the past three decades. In this letter-to-the-editor, the authors have summarized the practices for and challenges faced with hemophilia management in Thailand.
Thalassemia disease is a common inherited hemolytic anemia frequently found in several parts of the world, especially in the Mediterranean and some Asian countries. Besides the complications of secondary hemochromatosis from regular red blood cell (RBC) transfusion and increased gastrointestinal absorption of iron, thromboembolism (TE) is one of the common long-term complications of beta-thalassemia disease, particularly in patients with non-transfusion-dependent thalassemia (NTDT), which is commonly seen after the second decade of life. The risk factors of TE in beta-thalassemia disease including exposure of phosphatidylserine of abnormal RBCs, increase of platelet activation and aggregation, elevation of endothelial microparticles and increased endothelial activation, decreased nitric oxide (NO) secondary to hemolysis, rise of platelet count and nucleated RBCs after splenectomy, organ dysfunction caused by hemochromatosis, and thrombophilia such as natural anticoagulant deficiencies leading to hypercoagulable state. The understanding of the pathophysiology would result in effective prevention of this complication of beta-thalassemia disease.
Background/Objectives: Red blood cell distribution width (RDW) and mean platelet volume (MPV) are well-established prognostic biomarkers across various medical conditions. However, their role in predicting mortality among critically ill pediatric patients remains unclear. This study investigates the association between RDW, MPV, and 28-day mortality in pediatric intensive care unit (PICU) patients. Methods: This retrospective cohort study analyzed data from children aged 1 month to 18 years who were admitted to the PICUs at Chiang Mai University Hospital for ≥24 h between January 2018 and December 2022. The primary outcome was 28-day PICU mortality. A log-binomial regression analysis was conducted to assess the association of RDW and MPV with 28-day PICU mortality, adjusting for age, sex, mechanical ventilation, vasoactive drug use, continuous renal replacement therapy, and multiorgan failure. Results: A total of 580 PICU patients were included, 55.3% male, with a median age of 5.9 (IQR: 4.7–10.4) months. The 28-day PICU mortality rate was 9.8% (57/580). Respiratory failure and acute respiratory distress syndrome were the most common admission diagnoses (72.1%). Elevated RDW (≥15%) and MPV (≥10 fL) were independently associated with increased 28-day PICU mortality (adjusted RR: 2.73, 95% CI: 1.45–5.13 and 2.38, and 95% CI: 1.43–3.93, respectively). Both markers demonstrated high negative predictive values (RDW: 96.0% and MPV: 94.6%). Conclusions: Elevated RDW (≥15%) and MPV (≥10 fL) were independently associated with increased 28-day PICU mortality. These findings highlight their potential utility as accessible and cost-effective biomarkers for early risk stratification in critically ill pediatric patients.