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X linked agammaglobulinemia (XLA) is rarely reported from developing countries especially from South East Asia. It appears that × linked agammaglobulinemia is less common in certain ethnic groups. It is very uncommon in black people in USA and South Africa. In multiracial Malaysia we have documented five XLA in Malays and Indians but not in the Chinese that constitute about 31% of the population. First degree relatives afflicted with XLA or other primary immunodeficiencies occurred more often in our study. All showed lung involvement although the etiologic organisms involved were atypical, being Gram negative.

The awareness of primary immunodeficiency (PID) in Malaysia is still not forthcoming. Certain practical issues such as lack of clinical immunologists and specialized laboratory diagnostic facilities remain to be addressed. However, great efforts taken by passionate clinicians and scientists in the immunology networking have ascertained some prevalence. Despite the limitation, all suspected cases of PID are being properly investigated and competently managed. In this case report we highlighted the obstacles we faced in managing PID patients, particularly preparing for bone marrow transplant. This is the first transplanted case of chronic granulomatous disease in Malaysia, which emphasizes the importance of collaborative work to ensure further morbidities or mortalities are prevented.

We describe a Malay girl with disseminated cryptococcosis affecting the lungs, liver, lymph nodes and bones. The diagnosis was made by culture of the bone marrow. Tests of immune function showed that she was HIV-negative but the CD4 percentage was persistently low. Idiopathic CD4+ T-lymphocytopenia was diagnosed. The child died despite two courses of anti-fungal therapy.