Homozygous HbS in A Malay Patient: A Rare Variant of Sickle Cell Disease in Malaysia

@#Sickle cell disease in Malay ethnicity is uncommon, with few cases been reported only in Malaysian Indians. Detecting sickle haemoglobin in patients with osteoarticular manifestation is not as simple as those with haemolysis crisis, due to its extremely low incidence in this country. We hereby report a case of a 19-year-old Malay female who presented with a long-standing history of disabling movement of both hip joints, intermittent painful swollen right elbow, and chronic back pain. Imaging investigations revealed features of chronic osteomyelitis and avascular necrosis while blood investigations demonstrated features of mild normochromic normocytic anaemia and extravascular haemolysis. Further blood smear and haemoglobin analysis eventually confirmed the presence of homozygous sickle haemoglobin manifesting as sickle cell anaemia. Our case has highlighted the importance of prompt identification and thorough evaluation of the cause of anaemia in a patient with disabling chronic osteoarticular problem.

Severe Pulmonary Tuberculosis With Organizing Pneumonia: A Diagnostic Ambiguity

@#Pulmonary TB may present insidiously and ambiguously, leaving clinicians with a diagnostic dilemma. A 30-year-old lady with underlying spinocerebellar ataxia presented with progressive shortness of breath, prolonged cough with whitish sputum, loss of appetite and weight loss of 1-year duration. Physical examination showed a cachectic, tachypnoeic female with finger clubbing and coarse crepitations on lung auscultation. Chest radiograph showed bilateral air space opacities relatively sparing the upper zone. Contrast-enhanced CT thorax revealed bilateral cavitary necrotising consolidations, multiple scattered lung nodules with surrounding ground-glass opacities. After exclusion of alternative diagnoses, cryptogenic organizing pneumonia diagnosis was made. She had a rapid clinic improvement once steroid was started. TB polymerase chain reaction (PCR) from bronchoscopic bronchial washing eventually was positive. Anti-TB treatment was started, and oral steroid was slowly tapered down. Organizing pneumonia (OP) may complicate pulmonary TB. Diagnosing OP without lung biopsy requires a multi-disciplinary approach, taking into consideration all available evidences. Early steroid therapy is lifesaving and should be considered after thorough exclusion of alternative diseases.