Generalized Clostridial sepsis (Cl. Perfringens) with multiple organ abscesses in a patient with refractory very severe aplastic anemia (VSAA): haploid hematopoietic stem cell transplantation as the ultimum refugium
Abstract
The clostridial myonecrosis (gas gangrene), an infection caused by anaerobic microorganism Clostridium perfringens, or Clostridium septicum, is a life-threatening necrotizing infection affecting muscle tissue. The infection develops as a result of wound contamination with clostridial spores or thanks to hematogenous dissemination from the gastrointestinal tract to the muscles.
Early diagnosis and immediate initiation of adequate therapy are necessary. Our department has recently met with such a severe spontaneous generalized clostridial sepsis in a young immunocompromised man who has been treated for very severe aplastic anemia (VSAA) for a long time.
The initial examination showed multiple muscle and organ abscesses. Extensive, culture-focused (Cl.
Perfringens), combined wide-spectrum antibiotic therapy and current support of hyperbaroxy led to partial regression of abscesses and partial improvement of the clinical condition. However, continuing severe neutropenia (neutrophils <0,1 x 10exp9) thanks to VSAA, led to other infection complications (extensive invasive aspergillus pneumonia, sinusitis and VRE sepsis), when despite the complex antimicrobial therapy, there was a significant inflammation with no adequate resolution in the field of severe neutropenia.
Therefore, as the ultimum refugium, despite the difficult condition of the patient, a haploid transplantation of the hematopoietic cells from the patient's sister was undertaken (day +55 after admission). Immediate healing in all hematopoietic lines and the reconstitution of non-specific immunity resulted in complete regression of the inflammatory process and we could let the patient go home from hospital.