Bone Marrow Transplantation in X-linked T-B+NK- SCID patient with Pneumocystis carinii pneumonia

Transcription

1 Bone Marrow Transplantation in X-linked T-B+NK- SCID patient with Pneumocystis carinii pneumonia Lucie Šrámková 1, Aleš Janda 2 1 Dept. of Pediatric Hematology and Oncology 2 Dept. of Immunology University Hospital Motol, Prague

2 Family and personal history FH: negative PH: from 3rd gravidity, no full sibling (2nd gravidity death of female foetus in utero in 38th week of gest., stragulation by umbilical cord) birth weight 4000g, length 52cm adaptation after delivery normal BCG vaccination administered born on April 5, 2003

3 First 3 months of life without serious complications, only mild dermatitis around auricle

4 4 months of age petechia, maculopapulous rash cyanosis, hyposaturation, tachypnoe diarrhea, failure to thrive on chest X-ray susp. bilateral intersticial process CT of lungs without gross abnormalities serology respiratory viruses, chlamydia and mycoplasma negative no pneumocysts in gastric fluid hypogammaglobulinemia: IgG 2.57 g/l, IgA g/l, IgM g/l (after IVIG) Therapy: oxygenotherapy, corticosteroids 2mg/kg, atb clinical improvement, decreasing of the steroids

5 5 7 months of age diarrhea, decreasing of Ig levels observed by pneumologist in outpatient department 10 days after tappering of corticosteroids again tachypnoe, cyanosis, hyposaturation 2nd admission to the hospital

6 7 months of age hyposaturation, cyanosis, no effect of oxygenotherapy, no cardial abnormality hypogammaglobulinemia + T cell depletion: CD3+ 1.8%, CD4+ 0.3%, CD8+0.9% B cells 93.2%, NK cells 1.3% susp. CMV or Pneumocystis carinii infection in SCID patient T: gancyklovir, trimetoprim, corticosteroids 10mg/kg bolus, then 2 mg/kg/day respiratory insuficiency leads to artificial ventilatory support 24 hours after admission to our hospital

7 Immunology results January : (D-60) IgG<0,33 g/l, IgA<0,067 g/l, IgM=0,20 g/l CD3+ 7.5%, abs CD3-16,56+ 0 CD4+ 0,2%, abs. 0 CD8+ 3%, abs CD19+ 92%, abs SI 2, control 93

8 CT scan:severe intersticial lung process with atelectasis

9 DNA analysis no maternal engraftment X-linked T-B+NK- SCID (mutation of common gamma chain) analysis made by Genevieve de Saint Basile, Hopital Necker Enfants-Malades, Paris France

10 ICU: continuing ventilatory support with high FiO2 CMV quantitative PCR - negative 1st BAL not diagnostic open lung biopsy diagnosed pneumocystis carinii pneumonia with secondary alveolar proteinosis 2nd BAL massive positivity of Pneumocystis carinii planned to allosct D-11 sepsis Staph. epidermidis

11 Treatment trimethoprim+sulfamethoxazol, pentamidin inhalation (stopped after 6 days for susp. skin reaction) ciprofloxacin, meropenem, teicoplanin fluconazol GM-CSG 5ug/kg (5 weeks) IVIG substitution rifampicin, clarithromycin methyprednisolon (3 weeks)

12 Conditioning before BMT at the age of 10 months, 3rd month on ventilatory support ATG 10 mg/kg 4 times CsA 5mg/kg/day from D 3 methylpred. 1 mg/kg/day

13 Bone Marrow Transplantation March 10, 2004 bone marrow: 10x10 6 /kg CD34+cells MUD, 10/10

14 D+7 high temperature circulatory instability, supraventricular tachycardia CRP 160 coagulopathy colonization by multiresistant Acinetobacter and Klebsiella in airways Treatment: ciprofloxacin imipenem Xigris (recombinant activated protein C) trimethoprim+sulfamethoxazol rifampicin

15 D+12 clinically improved, blood cultures negative, PCR Pneumocystis c. negative D+25 extubation after 87 days of arteficial ventilation D+30 susp. skin GVHD of 2 nd -3 rd grade increasing of methylprednisolon on 2mg/kg/day

16 January : (D-60) Immunology results IgG<0,33 g/l, IgA<0,067 g/l, IgM=0,20 g/l CD3+ 7.5%, abs CD3-16,56+ 0 CD4+ 0,2%, abs. 0 CD8+ 3%, abs CD19+ 92%, abs SI 2, control 93 March : (D+13) CD3+ 83%, abs CD3-16, %, abs CD4+ 57%, abs CD8+ 27%, abs CD19+ 12%, abs ALC 1850

17 CT scan after BMT Rtg7.jpg

18 D+56

19 Thanks to: Dr. Dlask - Head of Pediatric ICU, University Hospital Motol and all staff for long term care Dr. Nohýnková for Pneumocystis carinii diagnosis Prof. Kodet for open lung biopsy results BMT team for fast donor search Dr. Čumlivská for CT findings