INTRODUCTION: Idiopathic CD4+ T-lymphocytopenia is defined by a CD4+ absolute count <300 cells, a CD4+ cell count <20% of total T cells, and a CD4+:CD8+ cell ratio of less than one on at least two successive laboratory determinations, together with confirmed absence of underlying HIV disease, and without a known cause of immunodeficiency or immunosuppressive treatment. Opportunistic infections, such as Mycobacterium kansasii pneumonia, cervical lymph node tuberculosis, disseminated tuberculosis and others usually represent the first clinical evidence of underlying primary CD4+ lymphocytopenia(1).
CASE PRESENTATION: A 35 year-old woman, 14 weeks pregnant after in vitro fertilization, presented with 1 month of fever, sweats, cough and sputum, after being treated with amoxicillin for urinary and sinus infection. She had a noncontributory physical exam, positive PPD and a micronodular dissemination on x-ray. Work-up showed mildly decreased CD4 count, negative HIV serology, negative sputum AFB smears and conventional bacterial cultures. Both transbronchial lung and bone marrow biopsies showed noncaseating granulomata. The patient developed sepsis and ARDS in need of noninvasive mechanical ventilation. She was empirically treated with a combination of anti-TB drugs, amphotericin B, and steroids; amphotericin was discontinued when fungal cultures were reported negative. She refused open lung biopsy. She lost her pregnancy. Eventually, sputum grew pansensitive M.tuberculosis and blood grew M. avium complex. On treatment, the patient improved and was discharged home; she did well on continuing anti-TB and anti-MAC treatment with complete clinical, bacteriologic, and roentgenologic resolution.
DISCUSSIONS: The cause of idiopathic CD4+ T-lymphocytopenia is still unknown. It is associated with a variable clinical spectrum,ranging from minimal or no symptoms to death from opportunistic infections or malignancies(in particular, lymphoproliferative disorders. After the 1993 tentative definition of CDC and WHO, multiple literature reports focused on small series or anecdotal case reports of T-lymphocyte immunodeficiency. This syndrome remains extremely rare but its exact frequency in the general population is unknown. An epidemiological and clinical study carried out in Senegal pointed out a 14.4% frequency of tuberculosis-associated CD4+T-lymphocytopenia among HIV-negative subiects (1).
CONCLUSION: Idiopathic CD4+ T-lymphocytopenia is rare but it should be considered in any patient with an otherwise unexplained opportunistic infection. We believe our case represents a good example of this entity. It is unique in that it occurred following in vitro fertilization. While a connection between that procedure and her infections is not clear, this unusual association may contribute to the understanding of the currently unknown pathomechanism.
DISCLOSURE: Farrakh Khawaja, No Financial Disclosure Information; No Product/Research Disclosure Information