Dr Ranil Perera describes the often diverse and complex haematological emergencies observed in primary care and how these should be managed

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Read this article to learn more about:

  • how to recognise the signs and symptoms of acute myeloid leukaemia
  • symptoms and causes of disseminated intravascular coagulation, sickle cell anaemia, priapism, and hyperviscosity syndrome
  • when cases should be considered medical emergencies.

 

Haematological conditions can present both insidiously or with patients in extremis. Often the latter is far easier to recognise and act on. This update is designed to provide primary care clinicians with an overview of haematological emergencies and the signs and symptoms not to be missed.

1 Acute myeloid leukaemia

Know the symptoms

People with acute myeloid leukaemia (AML) usually present with the symptoms of bone marrow failure, such as those related to neutropenia, thrombocytopenia, and anaemia.

Neutropenia can present with sepsis and patients may be haemodynamically compromised with tachycardia, hypotension, and fever at the point of assessment. Remember that septic patients on steroid treatment may be afebrile.

Although it is important to try to identify the source of sepsis, it is far more important to ensure that those people with suspected neutropenic sepsis are transferred to hospital urgently, with many secondary care guidelines recommending urgent broad spectrum antibiotics (usually tazocin and gentamicin) intravenously within 60 minutes.1

Other causes of neutropenia include chemotherapy, autoimmune conditions, and drugs (e.g. aminopyrine, quinidine, cephalosporins, penicillins, sulfonamides, phenothiazines, and hydralazine).2

The most common symptoms of anaemia are fatigue, reduced exercise tolerance, or even angina (in those with established ischaemic heart disease). There are cases of a myocardial infarction being the first presenting symptom of acute leukaemia in older patients.3

Thrombocytopenia may present with spontaneous ecchymoses, bleeding mucous membranes, epistaxis, or menorrhagia.3

Perform a thorough examination of bruising

When assessing any bruising, make sure to perform a thorough bruising examination and take a thorough history. The following elements of a history should raise concerns:4

  • bruising or bleeding that is seemingly spontaneous
  • bruising due to minimal trauma
  • bruising that occurs at multiple sites.

In children, also consider the possibility of non-accidental injuries.

Do not forget to consider the possibility of spinal cord compression in patients with known AML presenting with back pain, no matter how insidious.

Refer individuals for a very urgent full blood count

Adults and children should be referred for very urgent blood count tests (within 48 hours) if leukaemia is suspected.5 Box 1 (see below) details the symptoms that warrant further investigation in adults, including pallor, persistent fatigue, and unexplained bruising and bleeding. Children and young people should be referred for immediate specialist assessment for leukaemia if they have unexplained petechiae or hepatosplenomegaly, or symptoms similar to those seen in adults, such as pallor, persistent fatigue, and unexplained bruising and bleeding (see Box 2, below).5

Box 1: Referring an adult for suspected leukaemia5

When should I refer an adult with suspected leukaemia?

  • Consider a very urgent full blood count (within 48 hours) to assess for leukaemia in adults with any of the following:
    • pallor
    • persistent fatigue
    • unexplained fever
    • unexplained persistent or recurrent infection
    • generalised lymphadenopathy
    • unexplained bruising
    • unexplained bleeding
    • unexplained petechiae
    • hepatosplenomegaly (new NICE recommendation for 2015).

Box 2: Referring a child or young person for suspected leukaemia5

When should I refer a child or young person with suspected leukaemia?

  • Refer children and young people for immediate specialist assessment for leukaemia if they have unexplained petechiae or hepatosplenomegaly (new NICE recommendation for 2015)
  • Offer a very urgent full blood count (within 48 hours) to assess for leukaemia in children and young people with any of the following:
    • pallor
    • persistent fatigue
    • unexplained fever
    • unexplained persistent infection
    • generalised lymphadenopathy
    • persistent or unexplained bone pain
    • unexplained bruising
    • unexplained bleeding (new NICE recommendation for 2015).

2 Disseminated intravascular coagulation—consider the triggers

Disseminated intravascular coagulation (DIC) is an effect of the progression of other illness, usually those involving activation of systemic inflammation. This results in generation and deposition of fibrin, leading to microvascular thrombi in various organs, which contribute to multiple organ dysfunction syndrome.6 Consumption and subsequent exhaustion of coagulation proteins and platelets (from ongoing activation of coagulation) may induce severe bleeding.6

Conditions that are common triggers of DIC include:7

  • sepsis and severe infection
  • trauma
  • organ destruction (e.g. pancreatitis)
  • malignancy.

Disseminated intravascular coagulation is a life-threatening condition, and suspected cases should be sent via blue light to hospital.

3 Be aware of sickle-cell anaemia

Sickle-cell disease is a group of inherited, multisystemic conditions associated with episodes of acute illness and progressive organ damage.8 Most people affected are of African or African-Caribbean origin, although the sickle gene is present in all ethnic groups.8 Distortion and sickling of red blood cells occurs when they are exposed to low oxygen or acidaemia. As a result, these rigid cells can block small blood vessels, causing a lack of blood supply, inflammation, and activation of painful crises.8 Common areas affected are the periosteum, deep muscles, and bone marrow.

Although the trigger for these painful crises may not be apparent, they are commonly related to infection, exposure to cold, dehydration, exercise, stress, or alcohol.9

Acute painful crises should be treated as medical emergencies and be referred for admission if oral analgesia does not suffice or if there are serious complications.9

Patients with sickle-cell anaemia who present with pleuritic chest pain may be suffering with a crisis affecting their lungs. This is known as acute chest syndrome and is characterised by pleuritic chest pains, fever, abnormal chest examinations, and presence of new infiltrations on the chest X-ray. These patients should be admitted as an emergency and may need high dependency support.10

Aplastic crises occur when bone marrow function is impaired to the extent that haemopoesis is halted. Parvovirus is thought to be the causative agent. Patients usually present with a symptomatic anaemia, which should be treated with a prompt blood transfusion.11

4 Refer patients with priapism for more than 2 hours to A&E

Priapism is defined as a persistent penile erection that is either unrelated to sexual stimulation or continues for hours afterwards.

The causes are multifaceted and include:12

  • idiopathic
    • drugs:
      • anticoagulants
      • antidepressants
      • alpha-blockers
      • recreational drugs
      • drugs for intracavernous injection
      • sildenafil
      • testosterone
    • haematological disorders
    • metabolic disorders
  • trauma
  • tumours (primary or metastatic)
  • neurological disorders.

Priapism can be subdivided into two types:

  • Low flow—also known as ischaemic or veno-occlusive, low-flow priapism is often painful. If not treated it can persist for days, with long-term erectile dysfunction as a common outcome.13
  • High flow—high-flow priapism is also known as non-ischaemic or arterial priapism. It is usually not painful and is due to unregulated cavernous arterial inflow. It can occur after blunt perineal or penile trauma.14

Priapism, if not dealt with, can cause permanent erectile dysfunction. Initial management is to encourage patients to pass urine, trial warm baths, and exercise. However, if the priapism is ongoing for 2 hours they should present to A&E as soon as possible.14

5 Mucosal bleeding, visual changes, and neurologic symptoms suggest hyperviscosity syndrome

Patients with hyperviscosity syndrome typically present with mucosal bleeding, visual changes, and neurologic symptoms.15

Causes of hyperviscosity include:

  • Waldenström's macroglobulinaemia
  • multiple myeloma
  • leukaemia
  • polycythaemia
  • myeloproliferative disorders.

Be aware that, although the majority of patients will present obtunded with hypoxia, elderly people may be physiologically compensated and only report a mild headache.16

References

  1. Worcestershire acute hospitals NHS Trust. Guideline for the management of suspected neutropenic sepsis induced by cytotoxic therapy. NHS, 2015. Available at: www.worcsacute.nhs.uk/EasysiteWeb/getresource.axd?AssetID=9915&type=Full&servicetype=Attachment
  2. Braden C. Neutropenia. Available at: emedicine.medscape.com/article/204821-overview#a10 (accessed 26 January 2016).
  3. Seiter K. Acute myelogenous leukemia clinical presentation. Available at: emedicine.medscape.com/article/197802-clinical (accessed 26 January 2016).
  4. NICE. Bruising. Clinical Knowledge Summaries. NICE, 2010. Available at: cks.nice.org.uk/bruising#!topicsummary
  5. NICE. Haematological cancers—recognition and referral. Clinical Knowledge Summaries. NICE, 2015. Available at: cks.nice.org.uk/haematological-cancers-recognition-and-referral
  6. Levi M. Disseminated intravascular coagulation. Available at: emedicine.medscape.com/article/199627-overview (accessed 5 February 2016).
  7. Levi M, Ten Cate H. Disseminated intravascular coagulation. N Engl J Med 1999; 341 (8): 586–592.
  8. Sickle Cell Society. Standards for the clinical care of adults with sickle cell disease in the UK. Sickle Cell Society, 2008. Available at: bit.ly/20F42zM
  9. NICE. Sickle cell disease: managing acute painful episodes in hospital. Clinical Guideline 143. NICE, 2012. Available at: www.nice.org.uk/guidance/cg143
  10. Howard J, Hart N, Roberts-Harewood M, et al. Guideline on the management of acute chest syndrome in sickle cell disease. Available at: www.bcshguidelines.com/documents/Acute_Chest_Crisis.pdf
  11. Okpala I. The management of crisis in sickle cell disease. Eur J Haematol 1998; 60 (1): 1–6.
  12. Cherian J et al. Medical and surgical management of priapism. Postgrad Med J 2006; 82 (964): 89–94.
  13. Salonia A, Eardley I, Giuliano F. European Association of Urology guidelines on priapism. European Urology 2014; 65: 480–489
  14. Al-Qudah H. Priapism. Available at: emedicine.medscape.com/article/437237-overview#a1 (accessed 26 January 2016).
  15. Hemingway T. Hyperviscosity syndrome. Available at: emedicine.medscape.com/article/780258-overview (accessed 5 February 2016).
  16. Kwaan H. Hyperviscosity in plasma cell dyscrasias. Clin Hemorheol Microcirc 2013; 55 (1): 75–83. G