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Severe Clostridial Bacteraemia Complicated by Massive Haemolysis: A Case Report

A 54-year-old male teacher with a complex medical history, including Felty's syndrome and chronic neutropaenia, presented with acute abdominal pain and febrile neutropaenia. Initial management suggested pyelonephritis. However, he rapidly deteriorated, developing ARDS and septic shock due to Clostridium perfringens bacteraemia leading to massive haemolysis. Despite critical condition, he survived after extensive ICU care, including a laparotomy and significant blood product transfusion. This case highlights significant morbidities and rare complications associated with Clostridium infections.

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Severe Clostridial Bacteraemia Complicated by Massive Haemolysis: A Case Report

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  1. Blackwater fever Dr Josh Davis Staff Specialist Immunology & Infectious Diseases John Hunter Hospital

  2. Background • 54 yo married school teacher • Felty’s Syndrome • Diagnosed 1994. Splenomegaly. RF positive • RA currently quiescent • Chronic neutropaenia 2ry to Felty’s • No G-CSF or antibiotics • Nphils 0.3-0.5 since at least 1998 • GIH in 8/04. Settled spontaneously. Awaiting upper and lower endoscopies • No Regular Medications

  3. Presenting Illness • Well until 4 hours prior to presentation • At 18:00, crampy abdominal pain, loose stools (no blood), chills and rigors. • Went to MMH ED • O/E. T=40.1 degrees. Frank haematuria. Hypotensive 80 systolic. Nil else localising

  4. Initial Management • CXR clear, FBC 103/2.9/244, Bilirubin 153, other LFTs normal • ? Pyelonephritis in setting of febrile neutropaenia • Fluid resuscitation, Cefepime, Gentamicin

  5. Next 8 Hours • Haemoglobin plummeted • Developed respiratory failure with bibasal crepitations and bilateral new pulmonary infiltrates • ? Autoimmune haemolytic anaemia • ?Infection

  6. Progress • Transferred to JHH ICU • Rapidly developed ARDS, septic shock, DIC • Intubated, required noradrenaline infusion • Transfused for massive haemolysis • Blood cultures flagged positive

  7. Day 1 • Presumptive diagnosis: Clostridial bacteraemia with consequent massive haemolysis (Grew C.perfringens) • CT Abdomen revealed small liver collection • CT-guided pigtail drain placed in collection • Nearly exsanguinated into abdomen from liver puncture. Impossible to ventilate

  8. Day 1 • Transferred to OT for laparotomy “moribund” not expected to survive • On Nad 0.40mcg/kg/min, Vasopressin, Pressure Control Ventilation, 80% oxygen. BP 60-70 sys • APTT>100, PT 46, Fibrinogen 0.2 • 5 litres blood drained from abdomen. Liver packed

  9. Blood products first 4 days • Packed Cells • 46 units • FFP • 34 units • Cryoprecipitate • 52 units • Pooled platelets • 15 bags

  10. Progress • Survived against all odds • Developed ARF requiring CVVH then haemodialysis for 1 month • Discharged from ICU day 18, from hospital 46 • Received 6 weeks IV antibiotics (penicillin changed to metronidazole because of cholestasis) for liver abscess • Still awaiting scopes. Has dysphagia . . . . .

  11. Clostridial Bacteraemia 31/3/05 Josh Davis

  12. Clostridia - Microbiology • Anaerobic, spore-forming, gram positive rods. • 90 species exist – the most important in humans are: • C tetani – Tetanus • C botulinum – Gas gangrene • C difficile – Pseudomembranous colitis • C perfringens – see below • C septicum – bactraemia with colonic Ca.

  13. Clostridium perfringens - Microbiology • Ubiquitous in soil and faeces • Found in every soil sample ever tested except for the sands of the sahara • Found in the faeces of every vertebrate species tested • Originally called C.welchii • Secretes >12 exotoxins, most of which are lethal (to mice)

  14. Clostridium perfringens - Microbiology • Alpha - Toxin • AKA Lecithinase or Phospholipase C • Directly destroys RBC membranes, causing haemolysis • Enterotoxin • Food poisoning • Others • Haemolysins, DNAses, collagenase, protease, hyaluronidase • Spread through tissue and cause necrosis (gas gangrene)

  15. C.perfringens – Clinical Syndromes • Soft tissue infections • Simple wound infection (polymicrobial) • Crepitant cellulitis (does not invade healthy muscle) • Clostridial myonecrosis (gas gangrene) • Intraabdominal infections (see irrelevant aside) • Emphysematous cholecystitis • Enteritis necroticans • Typhlitis • Pelvic infections post-TOP • Primary bacteraemia • Food poisoning • Mild, no treatment required

  16. C. Perfringens bacteraemiaCase series – Rechner et al.1 • Rural US hospital all positive blood cultures 1990-1997 • Clostridia were 74 of 63,000 (0.12%) • C.perfringens most common, followed by C.septicum • 48% had underlying malignancy • Mortality was 58% • 52% were found to have a GI source • Only one patient had massive intravascular haemolysis 1- Rechner et al. CID 2001; 33: 349-53

  17. Massive haemolysis associated with C.perfringens bacteraemia • Literature review 1999, Alvarez et al.1 • 19 cases in entire world literature • 11/13 rapidly fatal (85% mortality) • Mean time from diagnosis to death=8 hours 1 –Alvarez et al. Massive hemolysis in Clostridium perfringens infectionHaematologica. 1999 Jun;84(6):571-3.

  18. C.perfringens bacteremia - summary • 1) Rare – (approx 0.1% of bacteraemias) • 2) Usually associated with underlying malignancy or debility • 3) Usual primary source is colonic lesion • 4) High mortality 40-60% • 5) Treatment=Penicillin, debridement • 6) Massive haemolysis very rare and almost always fatal within hours.

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