Blackwater fever

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Blackwater fever

• Dr Josh Davis
• Staff Specialist
• Immunology Infectious Diseases
• John Hunter Hospital

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Background

• 54 yo married school teacher
• Feltys Syndrome
• Diagnosed 1994. Splenomegaly. RF positive
• RA currently quiescent
• Chronic neutropaenia 2ry to Feltys
• 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

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Presenting Illness

• Well until 4 hours prior to presentation
• At 1800, crampy abdominal pain, loose stools (no
  blood), chills and rigors.
• Went to MMH ED
• O/E. T40.1 degrees. Frank haematuria.
  Hypotensive 80 systolic. Nil else localising

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Initial Management

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

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Next 8 Hours

• Haemoglobin plummeted
• Developed respiratory failure with bibasal
  crepitations and bilateral new pulmonary
  infiltrates
• ? Autoimmune haemolytic anaemia
• ?Infection

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Progress

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

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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

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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
• APTTgt100, PT 46, Fibrinogen 0.2
• 5 litres blood drained from abdomen. Liver packed

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Blood products first 4 days

• Packed Cells
• 46 units
• FFP
• 34 units
• Cryoprecipitate
• 52 units
• Pooled platelets
• 15 bags

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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 . . . . .

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Clostridial Bacteraemia

• 31/3/05
• Josh Davis

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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.

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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 gt12 exotoxins, most of which are lethal
  (to mice)

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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)

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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

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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
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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 death8 hours

1 Alvarez et al. Massive hemolysis in
Clostridium perfringens infection Haematologica.
1999 Jun84(6)571-3.
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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) TreatmentPenicillin, debridement
• 6) Massive haemolysis very rare and almost always
  fatal within hours.