Buku Haematology

Neutropenic sepsis

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Hypercalcaemia

Quick Tip; Venesection performed with a tourniquet in place can give an artificially elevated calcium

Hypercalcaemia can be a consequence of myeloma, some lymphomas and leukamias, and non-haematological malignancies. Presenting symptoms include;

  • Constitutional symptoms of lethargy and weakness
  • Polyuria and polydipsia
  • Abdominal pain, constipation and vomiting 
  • Spectrum of neurological impairment from low mood or confusion to drowsiness and coma
  • Rarely pancreatitis

Priorities of management are;

  • ABCDE assessment including ECG to check for cardiac arrhythmias
  • Identifying cause of hypercalcaemia from history if possible;
    • Review of medical history, and in the case of malignant hypercalcaemia, current disease therapies and the need to consider treatment of the underlying disorder to manage the source of hypercalcaemia
    • Review of current medications including calcium-containing compounds and thiazide diuretics
  • Blood tests including;
    • Full blood counts, renal function, bone profile, liver function tests
  • Risk starts to significantly increase of neurological or cardiac complications with a calcium level over 3mmol/L or any symptomatic hypercalcaemia
    • Start therapy with aggressive IV fluid resuscitation (often aiming for 3-4 litres/24 hours, monitoring closely for fluid overload)
    • Standard calcium-lowering therapies are pamidronate IV or zoledronate IV- see local guidance
    • If severe hyperclacaemia (over 3.5mmol/L) or significant cardiovascular or neurological sequelae, then calcitonin can be used rarely to bring down calcium more rapidly while awaiting the bisphosphonate effect- note risk of rebound hypocalcaemia therefore use with caution

Links:    BMJ  |  NICE CKS  

Spinal cord compression

Quick Tip; In any case of suspected malignant spinal cord compression immediate steroid therapy before imaging is required to minimise permanent neurological injury

Cord compression is an emergency that needs rapid action to prevent permanent neurological injury. Causes are most commonly solid organ malignancy metastases and myeloma, although it can also be caused by lymphoma or rarely patients with leukaemic infiltration of the cord can present as a cord compression syndrome.

Workup

  • History and examination
    • History of nature of back pain
    • Background of malignancy
    • Weakness of arms or legs
    • Sensory loss
    • Urinary incontinence or retention
    • Faecal incontinence
  • Examination for neurological injury
  • Investigations
    • Full blood count, bone profile, liver function tests, baseline coagulation screen (in case of neurosurgical intervention)
  • Steroid therapy (usually 16mg dexamethasone oral- monitor blood sugars) urgently if history and examination in keeping with cord compression
  • Urgent imaging - MRI is the gold standard

Short term management includes discussion with neurosurgery if a compressive lesion is found on MRI, depending on the appropriateness for the patient. Radiotherapy may be considered. Management of the underlying malignant cause is the definitive treatment.

Links:   BMJ  |  NICE Guideline 

Tumour lysis syndrome

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DIC

Quick tip; DIC will not resolve unless the underlying trigger is managed

Disseminated intravascular coagulation is a condition of gross over-activation of the coagulation pathway causing increased risk of clot formation and subsequent bleeding risk from consumption of clotting factors and platelets. This is most commonly triggered by trauma, infection, malignancy, severe inflammation such as pancreatitis and obstetric complications. A DIC-like syndrome can also be caused by acute promyelocytic leukaemia (APL), a subtype of acute leukaemia. DIC should be managed by the team caring for the patient in conjunction with haematology

Workup

  • History for potential trigger and plan for management thereof if possible
  • Clinical evidence of DIC such as unexpected or excessive bleeding or bruising, oozing from wounds or venepuncture sites
  • Baseline bloods;
    • Full blood count and blood film
    • Coagulation screen - Prolonged PT, APTT, low clauss fibrinogen, raised D-Dimer
    • Group and Save
    • Renal function
  • Those patients with established DIC will often need twice daily bloods
  • Critical care involvement where needed as mortality from DIC stems from end organ damage from micro-thrombi such as renal impairment and lung injury
  • While the underlying condition is managed, blood product support may be indicated in the context of abnormal clotting times and current bleeding, a risk of significant bleeding or planned procedures;
    • Correction of PT or APTT if significantly prolonged (Fresh frozen plasma-although large volumes of FFP are likely to be needed to 'normalise' clotting times which can contribute to fluid overload)
    • Platelet transfusion to keep count > 30-50 x109/L
    • Cryoprecipitate if fibrinogen less than 1.5g/L
    • These should all be guided by haematology advice and with regular patient review and repeat blood tests

Links:   Guideline  Blood Journal 'How I treat DIC'

TTP

Quick Tip; Not all elements of the classic 'pentad' are required to diagnose TTP

Thrombotic thrombocytopenic purpura is a form of microangiopathic haemolytic anaemia (MAHA) which is a syndrome of intravascular haemolysis of small vessels associated with end organ damage and significant morbidity and mortality. It is not a common condition but especially for haematologists, awareness and vigilance are critical as appropriate management will limit morbidity and mortality.

TTP arises either due to an inherited defect of, or acquired antibody to the ADAMTS13 enzyme which is used to breakdown large von Willebrand factor (vWF) multimers. Because of this large vWF complexes form in small vessels, cleaving red cells and causing haemolysis. Acquired causes include;

  • Malignancy
  • Medications such as ciclosporin and tacrolimus, and antiplatelet agents (not an exhaustive list)
  • Haematopoietic stem cell transplantation
  • Pregnancy

Due to this haemolysis and associated inflammatory response there is associated end organ damage. The classic pentad of Thrombotic thrombocytopenic purpura are;

  • Microangiopathic haemolysis
  • Fever 
  • Renal injury
  • Neurological symptoms such as confusion, drowsiness, seizures and coma
  • Thrombocytopenia
  • But not all must be present for a diagnosis to be made

Haematological investigations may show;

  • PT - Normal or increased
  • APTT - Normal or increased
  • Fibrinogen - Normal
  • D-Dimer - Normal or mildly elevated
  • Blood film - Schistocytes 

Diagnosis is made from a combination of the clinical features and seeing schistocytes (red cell fragments) on a blood film

Treatment needs removal of the precipitating cause and plasma exchange. This is the only definitive management of the condition and must be guided by haematology advice.

Links:   Guideline 

Sickle cell crisis

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