Trichobezoar masquerading as massive splenomegaly: Rapunzel’s syndrome revisited

Trichobezoars are usually formed due to ingestion of hair or hair-like fibres and present with a wide spectrum of clinical manifestations. We report a case of Rapunzel’s syndrome associated with trichotillomania in a 16-year-old girl who presented to our Haematology unit with complaints of fatigue, abdominal distention, and early satiety. Initial evaluation demonstrated anaemia, thrombocytosis, and a left hypochondrial mass suggestive of splenomegaly. However, ultrasound of the abdomen showed no hepatosplenomegaly and blood investigations were not suggestive of haematological malignancy. Not long after, the patient presented to the emergency department with suspected acute abdomen. Computed tomography of the abdomen revealed intraluminal gastric and jejunal masses causing small bowel obstruction. Emergency laparotomy confirmed gastric and jejunal trichobezoars, and subsequent psychiatric evaluation confirmed trichotillomania. Clinicians should consider trichobezoar in the differential diagnosis of abdominal pain and a non-tender ‘spleen-like’ abdominal mass

Spontaneous tumour lysis syndrome as a rare presentation of thymoma with peripheral blood lymphocytosis

Tumour lysis syndrome is common in haematological malignancies but is rarely reported in solid tumours. Peripheral blood lymphocytosis is an autoimmune feature of thymomas. We report a 63-year-old female who presented with a mediastinal mass, spontaneous tumour lysis syndrome and a leukoerythroblastic picture on peripheral blood film. Bone marrow aspiration and trephine biopsy ruled out haematological malignancy. Subsequent biopsy of the mediastinal mass confirmed thymoma. This is the first reported case of thymoma with peripheral blood lymphocytosis presenting with spontaneous tumour lysis syndrome. Clinicians are reminded that solid tumours may masquerade as haematological malignancies in the presence of peripheral blood lymphocytosis, hence careful clinical evaluation is needed to differentiate between the two diagnoses

The Sarawak Myelofibrosis (SaMy) experience: Demographics and outcome of myelofibrosis patients in Sarawak, Malaysia

Introduction: Myelofibrosis is a rare disease. There is currently no published data reporting the demographics and outcome of myelofibrosis patients in Malaysia. We aimed to study the demographics, clinical characteristics, and outcome of our patients in Sarawak. Materials and methods : This non-interventional, retrospective, and multi-center study was conducted on secondary data of medical records collected at four Sarawak Public Hospitals. All adult myelofibrosis patients diagnosed between January 2001 and December 2021 were included. Results : A total of 63 patients (male 31) with myelofibrosis were included—47 (74.6%) primary and 16 (25.4%) secondary myelofibrosis. Eleven had antecedent polycythaemia vera, whereas five transformed from essential thrombocythaemia. The combined annual incidence rate was 0.182 per 100,000 population. The period prevalence per 100,000 population over the entire study duration was 2.502. The median age was 59.0 years (33.0–93.0). Majority had high-risk (34/63(54.0%)) and intermediate-2 risk disease (19/63(30.2%)). JAK2V617F mutation was identified in 52 patients (82.5%), followed by CALR mutation in 6 (9.5%) and negative for both mutations in 5 (7.9%). Hydroxyurea was used as first-line therapy in 41/63 (65.1%), followed by interferon (8/63(12.7%)) and ruxolitinib (4/63(6.3%)). Out of 46 patients who received second-line therapy, 18 (39.1%) were switched to ruxolitinib and 9 (19.6%) to interferon. The median age of survival for overall patients was 6.8 years. The use of ruxolitinib in myelofibrosis patients showed a better overall 5-year survival compared to the no ruxolitinib arm, despite no statistical significance ( p  = 0.34). Patients who had good performance status had lower hazard of death than patients who had poor performance status (high-risk (95% confidence intervals): 0.06(0.013–0.239), p  < 0.001). Patients with intermediate risk disease had better overall survival compared to those in high-risk group (95% confidence intervals): 0.24(0.082–0.695), p  = 0.009). Conclusion : This registry provides a real-world overview of myelofibrosis patients in our state and highlights the key insight into the unmet clinical need