A 30-year-old Malay lady, with no previous or family history of bleeding, presented with severe gum bleeding 25 days post-partum. The factor VIII:c was 0.03 iu/ml with evidence of a slow-acting factor VIII inhibitor. Von Willebrand factor antigen (VWF:age) varied from less that 0.05 to 0.17 iu/ml, and there was absent ristocetin-induced platelet aggregation. Anti-nuclear and anti-DNA antibodies were present, but there were no other features of systemic lupus erythematosus. There was some clinical response to cryoprecipitate and tranexamic acid, and slight improvement with corticosteroid. Fifteen months later, the patient has no active bleeding problem, and her VWF-ag is increasing spontaneously. However, factor VIII:c is less than 0.01 iu/ml and her factor VIII inhibitor titre is still > 20 Bethesda units/ml.
Matched MeSH terms: Tranexamic Acid/therapeutic use
This is a case of a 62-year-old Indian man who was diagnosed with a rare type of lung
neuroendocrine tumour (NET) of atypical carcinoid (AC) subtype which comprises only 0.1%–
0.2% of pulmonary neoplasms. He initially presented to a private hospital in May 2018 with a
6-month history of chronic productive cough and haemoptysis. Chest X-Ray (CXR), CT scan,
bronchoscopy, biopsy and broncho-alveolar lavage were conducted. At this stage, imaging and
histopathological investigations were negative for malignancy. Diagnosis of bronchiectasis was
made and he was treated with antibiotic and tranexamic acid. Due to financial difficulties, his
care was transferred to a university respiratory clinic in June 2018. His condition was monitored
with CXR at every visit and treatment with tranexamic acid was continued for 6 months.
However, due to persistent haemoptysis, he presented to the university primary care clinic in
Dec 2018. Investigations were repeated in January 2019 where his CXR showed increased
opacity of the left retrocardiac region and CT scan revealed a left lower lobe endobronchial
mass causing collapse with mediastinal lymphadenopathy suggestive of malignancy.
Bronchoscopy, biopsy and histopathology confirmed the presence of NET. Although the Ki-67
index was low, the mitotic count, presence of necrosis and evidence of liver metastases
favoured the diagnosis of AC. A positron emission tomography Ga-68 DONATOC scan showed
evidence of somatostatin receptor avid known primary malignancy in the lungs with suspicions
of liver metastasis. He was subsequently referred to the oncology team and chemotherapy was
initiated. This case highlights the challenge in diagnosis and management of patients with AC.
Physicians ought to be vigilant and have a high index of suspicion in patients who present with
persistent symptoms on multiple visits. Early diagnosis of NET would prevent metastasis and
provide better prognosis. Continuous follow-up shared care between primary care and
secondary care physicians is also essential to provide ongoing psychosocial support for
patients with NET, especially those with metastatic disease