Pure white cell aplasia (PWCA) is a rare manifestation of thymoma. It is characterized by agranulocytosis with absent myeloid precursors in the bone marrow and normal hematopoiesis for other cell lines. Here we describe a 65-year-old female patient who presented with three days of fever and night sweat. Chest CT revealed an anterior mediastinal mass. A biopsy of the mass confirmed a diagnosis of thymoma mixed type A and B2. The patient developed a severe neutropenia, and her bone marrow revealed significantly decreased neutrophil-lineage cells, rare to absent B cells, and defective T cells, consistent with PWCA. Following thymectomy, a complete resolution of PWCA was achieved via multimodality therapy of intravenous immunoglobulins, granulocyte colony-stimulating factor, and immunosuppressant. This report highlights the care complexity regarding treatment choices and decision to perform thymectomy in patients presenting with PWCA.
Background
Snakebite‐related injury is a serious public health issue. In Cambodia, it is estimated that up to 21,500 cases of envenoming occurs from snakebites annually. Musculoskeletal disability is a major long‐term complication associated with the injury. In this study, we aim to describe surgical management and rehabilitation in snakebite‐related musculoskeletal injuries at Children's Surgical Centre, Phnom Penh, Cambodia.
Methods
We conducted a retrospective case series analysis of patients with snakebite‐related injury who were treated between January 1, 2002 and December 31, 2018. Surgical patients were divided into the early and late presenting groups (= < one year vs. > one year, respectively) based on their time interval from snake bite to time of presentation.
Results
There were 88 patients who presented with snakebite‐related musculoskeletal injury during the cohort study period. Majority of them were male (n = 62, 71%) and had a median age of 24 years old (IQR 17–44). The injuries were all in the upper and lower limbs though lower limb injury was more common in female patients (81% vs. 48%, Fisher's test p = 0.005). The median time interval from snakebite to time of treatment was 3 years (IQR 3 months–11 years). In this study, 65 patients received surgical interventions. An ulcerated wound was the most common symptom among the early presenting group (78% vs. 24%), while scar contracture was most common among the late group (76% vs. 22%) (Fisher's test p < 0.0001). For management, surgical debridement was the most common primary intervention for the early group (52% vs. 19%), and contracture release was the most common for patients in the late group (62% vs. 15%) (Fisher's test p = 0.0004). Overall, the postoperative complication rate was highest in the late presenting group (34% vs. 3%, Fisher's test p = 0.005).
Conclusion
More than half of the patients presented with musculoskeletal injury require surgical correction. Our study demonstrated that scar contracture is the most common complaint among the late presenting group and is associated with high postoperative complication rate.
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