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Porter J, Taher A, Mufarrij Aet al., authors; Eleftheriou A, Angastiniotis M, Telfer P, editors. Emergency Management of Thalassaemia [Internet]. Nicosia (Cyprus): Thalassaemia International Federation; 2012.

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Emergency Management of Thalassaemia [Internet].

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Chapter 4PROBLEM - RELATED MANAGEMENT

4.1. SEPSIS

Consider possible causes and investigations.

  • Infections are typically bacterial and are often rapidly progressive and potentially fatal.
  • Infections are the second cause of death in TM, after cardiac complications.
  • Thalassaemia patients should be treated as if immunocompromised if they have a fever.

Common organisms involved include:

  • Klebsiella, which may cause septicaemia and shock
    A variety of other gram negative organisms
    Cerebral abscesses
    Yersinia enterocolitica, more commonly occurring in patients on desferrioxamine. Presents with fever, abdominal pain, diarrhoea lymphadenopathy. May mimic acute appendicitis. Difficult to detect and the microbiology laboratory must be alerted to this possibility when receiving samples of stool or blood.
  • A variety of gram positive encapsulated organisms may cause infection, particularly in splenectomised patients.

Common foci of infection include:

  • Renal tract - UTI, renal stones
  • Respiratory tract
  • Abdomen
    1. cholecystitis
    2. colitis, appendicitis (but consider first excluding yersinia)
  • Indwelling lines/catheters
  • Cerebral (abscess)
  • Heart (myocarditis and endocarditis)
  • Septicaemia without a focus – consider splenectomy and severe iron load.

To address Sepsis follow EGDT guidelines (Early Goal Directed Therapy).

[Rivers E et al NEJM 2001, 345(19)]:

  • Provide early fluids (avoid overload since many patients have cardiac involvement due to iron toxicity – each case must be assessed according to cardiac status)
  • Administer early antibiotics
  • Monitor parameters e.g. mean arterial pressure, central venous pressure, lactate
  • Provide inotropic agents if indicated

Antibiotics

When giving antibiotics in the emergency unit to a thalassaemia patient, care should be taken to always cover for pneumococci, Haemophilus influenzae and meningococci if splenectomised.

Acute septicaemic patients should be covered for a range of gram negative pathogens including Klebsiella (ciprofloxacillin is often effective).

Should Yersinia - an intracellular organism - be suspected, ciprofloxacillin or trimethoprin are usually effective.

Local antibiotic sensitivities should be also considered and an infectious disease specialist may need to be consulted.

If there is an indwelling catheter, Teicoplanin or Vancomycin are the antibiotics of choice, especially if the patient has recently been hospitalised, requiring cover for MRSA.

If a patient has a fever always admit, unless you are advised by the treating thalassaemia specialist that there is no need to do so.

SUSPECTED INFECTION/FEVER

Always admit except for cases cleared by thalassaemia specialist.

Send: cbc, chem, urine

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FEVER:FOCUS STILL UNCLEAR

Always admit except for cases cleared by thalassaemia specialist.

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4.2. ANAEMIA

Consider possible causes and investigations.

Patients with TM should be on a regular transfusion program to keep Hb >9.0g/dl (9.5 – 10 g/dl) - see TIF Guidelines for the Clinical Management of Thalassaemia 2nd Edition Revised (2008).

Values below this may result from:

  • delayed transfusions
  • delayed transfusion reactions
  • bleeding (e.g. GI from peptic ulcer)

Patients with TI have a wide range of steady state Hb (6-9g/dl).

  • Ask the patient, family or treatment centre the patient’s usual Hb level. A sudden decrease in Hb may result from
    • - parvovirius infection (B19) (may lead to Aplastic crisis)
    • - other acute infections
    • - delayed transfusion reactions
    • - enlarged spleen (subacute)
    • - folate deficiency
    • - G6PD deficiency

Transfusion should only be given as a life-saving measure, for example in cases of severe bleeding or anaemia with instability. Before embarking on transfusion:

  • Consult with the treating physician and blood bank specialist
  • Ensure that the Hb is significantly lower than the steady state
  • Establish patient history of transfusion reactions
  • Request extended red cell phenotype

TM patients usually receive blood <10 days old that is at least Kell negative and matched for extended rhesus phenotype - refer to TIF Guidelines for the Clinical Management of Thalassaemia 2nd Edition Revised (2008).

4.3. DYSPNOEA

Consider possible causes and investigations.

  • Heart failure resulting from cardiomyopathy. This has a potentially rapid progression from severe to fatal.
    • - Check BP, pulse rhythm (associated dysrhythmia is common), JVP, liver congestion
    • - Abdominal pain may be presenting feature of congestive cardiac failure (CCF)
    • - Peripheral oedema
    • - May be precipitated by myocarditis, sepsis, poor recent chelation or poor chelation adherence
    • - Consider immediate iv DFO infusion unless contraindicated (allergy, suspected Yersinia infection) – 40-50mg/kg day as a continuous iv infusion dissolved in 100-500ml of saline
    • - Ask if a recent cardiac MRI has been done
    • - Consider MRI or echocardiogram if MRI is not available.
  • Dysrhythmia - patients with myocardial iron loading (present or past) are at increased risk of a variety of dysrhythmias, which can precipitate heart failure.

    Check ECG – consider 24h monitoring.

  • Pericarditis (see section 4.3) - occasional complication of iron-mediated cardiomyopathy.
    • - listen for pericardial rub (inflammation) or faint sounds (effusion)
    • - consider echocardiogram.
  • Pulmonary embolism
    • - thalassaemia patients, particularly TI, are susceptible.
  • Pulmonary hypertension
    • - thalassaemia patients, particularly TI, are susceptible.
  • Anaemia (acute - see section 4.2)
  • Rib micro-fractures and splinting

4.4. OEDEMA

Consider possible causes and investigations.

  • Congestive heart failure (see 4.3)
  • Venous thrombosis
    • - red cells are prothrombotic, so more likely in TI.
    • - may present with typical leg DVT.
    • - other sites include mesenteric blood vessels.
    • - multiple small pulmonary emboli (PE).
  • Hypoalbuminemia
    • - renal protein loss - check urine protein.
    • - liver failure – check LFTS.
  • Leg ulcers more common in TI - examine the legs.
  • Acute renal failure

4.5. CHEST PAIN

Consider possible causes and investigations.

  • Pulmonary embolism
    • - may be a single, large embolus but often repeated small emboli.
    • - consider requesting d-dimer, high resolution CT.
  • Rib (micro) fracture
    • - palpate chest wall - pain on gentle pressure.
  • Pericarditis
    A complication of iron overload.
    • - listen for heart sounds, do ECG, echocardiogram.
  • Congestive heart failure
    May present as abdominal rather than chest pain, caused by distension of the liver capsule.
  • Acute coronary syndrome
    Very unlikely but should be considered.

4.6. ABDOMINAL PAIN

Consider possible causes and investigations.

  • Cholecystitis
    • - pigment gallstones are common.
    • - consider abdominal ultrasound.
    • - if obstructed (bilirubin in urine, ± ↑ Alk Phos), consider ERCP.
  • Yersinia infection
    • - iron-loving organism more commonly seen in patients receiving desferrioxamine.
    • - requires specific culture conditions for diagnosis from blood or faeces. contact microbiology for Yersinia culture procedures.
    • - desferrioxamine should be stopped until diagnosis is confirmed or excluded
  • Portal vein thrombosis
    • - more commonly seen in TI and splenectomised TM patients.
  • Mesenteric infarction
    • - more commonly seen in TI and splenectomised TM patients.
  • Renal stone
    • - common in TM due to hypercalciuria.
    • - consider renal ultrasound and urine microscopy (red cells).
  • Peritonitis
    Especially in splenectomised patients.
  • Gastroenteritis
  • Acute appendicitis
    Should be considered after exclusion of Yersinia.

4.7. HEADACHES

Consider possible causes and investigations.

Clues in patient history: consider duration, whether recurrent, whether increasing in severity (“the worst headache I have ever had”), whether onset is sudden and whether the episode is accompanied by vomiting.

Clues in physical examination: check for pyrexia, neurological signs, papilloedema, altered level of consciousness, stiff neck.

Investigations: computerised tomography (CT) with contrast (more common in hospital settings and more rapid than MRI), although MRI is more sensitive to cerebral abscesses

  • Cerebral abscesses Klebsiella may be the causative agent. Cerebral abscess commonly presents as headache, fever and/or neurological features, but often with a non-specific prodromal phase.
    Iron overload, especially in TM, increases patient susceptibility to infection with Klebsiella and other iron-loving micro-organisms. A high index of suspicion should therefore exist to confirm or exclude diagnosis with these pathogens.
  • Meningitis - especially if the patient is splenectomised.
  • Sinusitis - more commonly seen in TI due to distortion of sinuses (extra-medullary erythropoiesis).
  • Otitis media - more commonly seen in TI patients due to distortion of sinuses.
  • Extramedullary haemopoietic mass - rarely occurs intra-cranially.
  • Drug-related headaches
    (e.g. interferon, used in the treatment of chronic HC and/or HB).
  • Consider other common non-thalassaemia related causes. e.g. migraine

HEADACHES

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4.8. SYNCOPE AND ALTERED LEVEL OF CONSCIOUSNESS

The patient with syncope or pre-syncope symptoms must be admitted for investigation.

Consider causes include the following.

  • Tachydysrhythmias, such as atrial fibrillation or ventricular tachycardia.
    • - may result from iron-mediated cardiomyopathy, even after iron has been removed with intensive chelation.
    • - may be exacerbated by electrolyte disturbances such as hypocalcaemia.
    • - consider 24h motoring.
  • Repeated pulmonary emboli - small pulmonary emboli are quite common in TI but may also occur in TM (red cells are prothrombotic in thalassaemia).
  • GI bleed
    • - drugs - e.g. non-steroidal (also take into account side-effects of iron chelation drugs, such as upper GI ulceration).
    • - varices - some older patients have varices secondary to portal hypertension.
  • Postural hypotension or vasovagal syndrome
    • - many TM patients have low resting blood pressure, and inadequate postural response may cause syncope
  • Brain abscess (see section 4.9)
  • Hypovolaemia (fluid loss, acute severe anaemia)

In all cases call an expert, get clearance from next of kin and admit.

4.9. NEUROLOGICAL COMPLICATIONS

Causes to consider.

Focal deficits

  • Extramedullary haematopoiesis - may occur anywhere but often results in spinal deposition with weakness, paraesthesia and sensory loss in the legs. Difficulty in bladder control or urgency may also be a presenting feature.
  • Cerebral abscess - bacteraemia may result in central brain abscess, often reported with Klebsiella pathogen. Symptoms may be non-specific, such as malaise and headache. Brain MRI is recommended where available.
  • Meningitis - more likely to be seen in splenectomised patients
  • Spinal thrombosis - rare complication which presents with acute motor or sensory feature, often with a neurological dimension. MRI is the initial investigation of choice.

Other neurological complications

  • Deteriorating hearing or tinnitus.
    • - known side-effect of desferrioxamine overdosing - consider iron chelation regimen.
    • - stop desferrioxamine and seek audiometry assessment.
  • Change in visual acuity or colour vision.
    • - known side-effect of desferrioxamine overdosing - consider iron chelation regimen.
    • - stop desferrioxamine and seek electro-retinography.
    • - consider diabetic retinopathy, which may also result in sudden visual deterioration.

FOCAL NEUROLOGICAL DEFICIT

Upper Motor Neurone (UMN)

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4.10. BACK PAIN

Causes to consider

  • Osteoporosis
    • - reduced bone mineral density of the spine is reported in around 50% of adult thalassaemia patients.
    • - causes micro-fractures and full compression fractures of the vertebral bodies.
  • Degenerative changes of the intervertebral disc - common cause of back pain.
    More common in the lower thoracic and upper lumbar spine (compared to non-thalassaemic patients, more commonly affected in the two lower lumbar discs).
  • Extramedullary haemopoietic masses
    • - may cause spinal cord compression
    • - neurological signs of cord compression more likely to be present than pain.

Flattening of the vertebral bodies (platyspondyly) in the lumbar and thoracic regions.

Investigations

  • - exclude neurological deficit on examination
  • - spinal X-ray and MRI (best method of assessing extramedullary haematopoiesis and also disc degeneration).

ACUTE BACK PAIN

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4.11. TRAUMA

In thalassaemia, be alert to possible:

  • Splenic rupture
  • Bone fracture
    • - occurs with relatively minor trauma
    • - sites include:
      • - ribs
      • - spine
      • - hips
      • - metatarsals

Adhere to Acute Trauma Life Support (ATLS) guidelines

(see www.facs.org/trauma/atls/information.html)

Early involvement of general surgeon.

Special transfusion requirements (see section 4.2).

© 2012 Thalassaemia International Federation.

All rights reserved.

The publication contains the collective views of an international group of experts and does not necessarily represent the decisions or the stated policy of the Thalassaemia International Federation.

Bookshelf ID: NBK190437

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