Abstract

Patient is a Chinese girl of 13 years. She had fever, weakness, headache, and was hospitalized on Feb 20th, 2003. There were 0.89 leukemic monocytes in her bone marrow smear at diagnosis. She was diagnosed acute monoblastic leukemia (M5b) according to FAB classification. The immunophenotyping of bone marrow cells showed CD13 0.579, CD15 0.289, CD33 0.78, cMPO 0.27. After the second remission, she underwent hyplo-identical, ABO-matched combination transplantation of bone marrow and peripheral blood stem cells of her donor mother with T-cell deletion in Aug. 2005. Her hemapoiesis recovered at +10d after transplantation. The while complete blood cell count recovered at +31d when the TCR-PCR DNA gene map was showed the chimera. The rejection appeared at +60d. Her blood picture showed Hb 63g/L, Plt 1.0×109/L, WBC 0.4×109/L. The bone marrow picture showed hypo-cellular as same as in severe aplastic anemia. The TCR-PCR DNA gene map showed the 3/16 locus of recipient. The schedule of treatment of delayed rejection was the combination of Cyclosporin A (CsA), Mycophemolate Mofeil (MMF) and Methylprednisolone (MP). She boosted the G-CSF-primed peripheral mononuclear cells of her mother. The cell dose was 4.3×108/kg without T-deletion. Then she received the stimulation of G-CSF 250 ug daily. One week later, she suffered from Herpes Zoster Virus (HZV) infection. There was characteristic HZV varicella in full right side of head, right face, right eye, and right hand with high fever, malaise. She felt severe postherpetic neuralgia. Only morphine or remain acupuncture can relieve her severe neuralgia. She had also severe pancytopenia, gastrointestinal bleeding. We had to transfuse backed red cell and platelet concentration every week. She received first TPE at +130d and second TPE at +137d after transplantation. Dramatic results were gotten after the two TPE. After TPE she did not need transfusion again. After the third TPE, she was discharged then had treated small dose of CsA for half year. The STR-PCR DNA gene map showed the gene type from her mother. At +311d she suffered from chronic graft versus disease (cGVHD). The skin lesion involved her whole body skin about was about 50% body surface area. The effect had not received after treatment with TPE. The cGVHD had not controlled after the basic treatment with CsA or Sirolimus (FK506), MMF, MP, and Thalidomide 100–400 mg/d. So we added with ultraviolet-B irradiation (UV-B) therapy with dose of narrowbank UV-B, twice a week. The lichenification planum was disappearing. The mechanism of TPE on delayed rejection in ABO-unmatched, allogeneic stem cell transplantation has been clear. But, it is still not clear in ABO-matched and hyplo-identical allogeneic stem cell transplantation. It may be cause humoral rejection as same as ABO-incompatible stem cell or solid organ transplantation. The antibodies have resulted from hyplo-identical allogeneic stem cell of her mother. The mechanism of cGVHD results from donor T-lymphocyte activated and involves skin, liver, gut and so on. It is effective of treatment of cGVHD with immune inhibitor drugs but with TPE. Recently reported ECP is effective because the method result in suppressing the activated T-lymphocyte in recipient.

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