Idiopathic CD4+ T-Lymphocytopenia — One Case Report from China

The patient was a 55-year-old femal staff who presented with gingivitis in July 2009. The result of blood routine test indicated WBC was 2.6×10⁹/L, neutrophil count was 0, lymphocyte count was 2.1×10⁹/L, hemoglobin count was 119g/L, and PLT count was 342×10⁹/L. In addition, the ratio of heterotype lymphocytes was 8% in blood smear. Lymphocyte subsets analysis showed that CD3+T lymphocytes took up 57%, CD3+ CD4+ T lymphocytes took up 12% (count 250 per cubic millimeter), CD3+ CD8+ T lymphocytes accounted for 40% (count 840 per cubic millimeter), NK cells took up 40% and the ratio of CD4/CD8 and CD4/CD3 were 0.3 and 0.21 respectively. The patient’s liver function was abnormal and serological testing of HIV, EBV and CMV was negative. Enterobacter cloacae was cultured from the secretions of gum. Bone marrow smear showed active proliferation of cells with lymphocytes took up 32% which consisted of T cells with normal morphology mainly. The chromosome of the patient was normal. Ultrasound test showed enlargement of the lymph nodes in the neck with normal echo. She responded well to treatment with antibiotics, granulocyte colony-stimulating factor and supportive care. In September 2009, her CD4+T lymphocyte count was still very low (440 per cubic millimeter) and the ratio of CD4+ /CD3+ lymphocytes was 0.28. Then she started intramuscular injection of thymopentin 1mg daily for one month and decreased the frequency from twice or three times a week to once every ten days. She occasionally felt swelling pain of her gum and lymph nodes. CD4+ T lymphocyte count was 500~900 per cubic millimeter and CD8+ T lymphocyte was 800~1400 per cubic millimeter. In the Junes of 2010 and 2013, her ratio of CD4/CD3 unprovoked declined (to 0.17) with mild hepatic dysfunction. After increasing the injection frequency of Thymosin α1,her CD4+lymphocyte count increased (less than 740 per cubic millimeter). She is asymptomatic at this writing.

During the course, gene rearrangement of TCRγ-VJ1and TCRγVJ2 was positive in June 2010 and turned negative in June 2013. Results of bone marrow smear and biopsy were normal and multiple serologic testing of HIV, EBV and CMV was negative. Her auto-antibodies were tested negative too. A PET-CT scan found small lymph nodes on both sides of her neck with average SUV value of 2.3 in June, 2013. She was not homosexual. She had constipation for many years while endoscopy of the stomach and intestine was normal. She had no history of taking immune-suppressive drugs or blood transfusion except for hysteromyomectomy in 2002. She had no special family history.

According to the American CDC’s diagnostic criteria in 1992, this patient was diagnosed with idiopathic CD4+ T-lymphocytopenia. The disease is extremely rare, only two cases reported so far in China. Doctors in the clinical work in patients with recurrent infections especially opportunistic infections should do routine lymphocyte immune classification which can improve the diagnostic rate of this disease. The prognosis of the disease is mostly good. Thymopentin or thymosin may improve the degree of immune deficiency and reduce infections.