Haematological Emergencies Pieter de Witt 18 Oct 2014

Overview � Malignant spinal cord compression

� Hypercalcaemia

� Superior Vena Cava syndrome

� Neutropaenic fever

� Extreme Cytosis

Malignant Spinal Cord Compression

MSCC Spinal cord

MSCC Importance / Urgency � Neurological status at Rx

initiation � Most important prognostic

factor

MSCC Clinical presentation � Pain

� Motor findings

� Sensory findings

� Bladder and Bowel dysfunction

� Ataxia

MSCC Imaging � Plain X-rays

� Magnetic Resonance Imaging – preferred

� CT Myelography

� Image the whole spine Multi-level deposits are present in 1/3 of patients with MSCC

MSCC Management � Goals � Pain control � Preservation or improvement of neurological

dysfunction � Avoid complications

MSCC Management cont… � Specific therapy

� “Glucocorticoids” � Decompression � Surgery � Radiation � +/- Systemic chemotherapy

� Supportive management � Pain control � Bed rest � Anticoagulation � Prevent constipation � Pressure care

MSCC Prognostic factors � Neurological status prior to therapy � Histology

� Extent of disease � Visceral or bony metastasis

� Aggressiveness of the tumor � Interval between initial diagnosis and MSCC < 15mo

MSCC Key points � Early recognition

� Image the whole spine

� Potentially curable condition

Hypercalcaemia

Hypercalcaemia Causes � Hyperparathyroidism – most common

� Malignancies � Osteolyitic metastasis � PTHrP � 1,25 dihydroxy vitamin D

Higher incidence of above conditions to occur in the same patient

� Other rare causes…

> 90%

Hypercalcaemia Clinical presentation � Depends on: � Degree of hypercalcaemia � Rate of onset � Individual sensitivity

Stones, bones, abdominal groans & psychic overtones

Presenter

Presentation Notes

Polyuria – Nephrogenic Diabetes Insipidus Impaired urinary concentration in the distal convoluted tubule & collecting duct Downregulation of Aquaporin 2 channels, deposition of Calcium in the medulla Calcium sensing receptors Dehydration – Polyuria with anorexia, nausea and vomiting

Hypercalcaemia Management

AIMS Identify and treat the cause Lower serum calcium

Hypercalcaemia Management

Targets

1. Dilute 2. Excrete 3. Influx 4. Remove

Hypercalcaemia Key Points � 2 main causes

� Fluids, fluids, fluids, fluids +/- diuretics

� Bisphosphonates requires 72hrs to work

Superior Vena Cava Syndrome

SVC Syndrome

� SVC obstruction � Compression, invasion and/or thrombosis

� Causes � Pre-antibiotic era � Infective causes

� Post-antibiotic era � CANCER = 90%

� Present era � Benign causes = 20 – 40%

SVC Syndrome Clinical manifestations

Malignant SVC Syndrome Management

� PAST – was considered a potential life-threatening medical emergency requiring immediate Radiotherapy

� NOT ANYMORE,…. UNLESS � Stridor � Central airway obstruction or � Laryngeal oedema

� Coma from cerebral oedema

Stent placement and Radiotherapy

Malignant SVC Syndrome Management

� Principles � Diagnose and treat the underlying cause � Alleviate symptoms

Malignant SVC Syndrome Key Points � NO MORE emergency

radiotherapy unless…

� Urgent diagnostic work-up required

� Treatment directed at the cause

Neutropenic fever

Neutropenic fever Definitions � Definitions � Fever � Single oral temp > 38.3°C � Temp > 38.0°C sustained for > 1hr

� Neutropenia = ANC < 2 (1.5) � Severe Neutropenia = ANC < 0.5 � Profound Neutropenia = ANC < 0.1

Neutropenic Fever Risk � Low risk � Severe neutropenia lasting ≤ 7 days � No comorbidities � No significant liver or renal dysfunction

� High risk � Expected severe neutropenia lasting > 7 days � Comorbidities � Significant organ dysfunction Regardless of the duration of neutropenia

Neutropenic Fever Pathogenesis � Chemotherapy � Antineoplastic effect ALSO cause � Myelosuppression � Neutropenia

� Mucositis � Translocation of GIT microorganisms

� Tumor breeches natural host defenses � Obstruction � Ulceration / invasion

Neutropenic Fever Epidemiology � Only 20 – 30% of patients – identifiable infectious source

� Endogenous flora – responsible for 80% of infections

� Bacteria – most frequent infectious cause � Gram negatives (esp Pseudomonas) – most serious � Gram positives � S epidermidis most common (much less virulent) � S aureus & Viridans Streptococci – more serious gram positives

� Anaerobes – infrequent pathogens � Polymicrobial infections - infrequent

Neutropenic Fever Epidemiology � Fungal infections – esp in high risk patients � Duration of neutropenia � Severity of neutropenia � Prolonged antibiotic use � Number of chemotherapy cycles

� Candida & Aspergillus & Mucormycosis

� Viral infections � Herpes viruses – HSV, VZV, CMV, EBV

Neutropenic Fever Management

CRITICAL TO RECOGNIZE !

� Early initiation of empiric systemic antimicrobials � Within 60min of presentation! � Delayed antibiotics – mortality as high as 70%

� Cultures (esp blood) prior to antibiotic initiation

Neutropenic fever Key points � Assume – Gram negative sepsis

� Culture

� Antibiotics within 60min of arrival

Extreme Cytosis White cells

� Hyperleukocytosis

� Leukostasis

Lymphoid vs Myeloid

Leukostasis Clinical presentation

� Pulmonary

� Neurological

� Fever

Hyperleukocytosis Management � Cytoreduction � Chemotherapy � Hydroxyurea - 50-100mg/kg (2 to 4g q12hrly per os) � Leukapheresis

� Supportive care � Blood viscosity � Red cell transfusions, diuretics

� Coagulopathy � Platelet transfusions and DIC

� Tumor lysis prophylaxis � Fluids, Allopurinol

� Prognosis

Hyperleukocytosis Key points � Leukostasis – clinical diagnosis

� Requires urgent cytoreduction

� Supportive care

Extreme Cytosis Red cells � Definitions � Hct > 48% (♀) or > 52% (♂) � Hb > 16,5 (♀) or > 18,5 (♂)

� Relative polycythemia

� Absolute polycythemia � Primary � Secondary

Erythrocytosis

Erythrocytosis Management � Control the underlying cause

� Phlebotomies � No clear guidelines with threshold Hct levels � Suggestions: � Considered in hypoxic conditions � Limited phlebotomy � Isovolaemic venesections � Avoid iron deficiency � Iron replacement used cautiously

� Other � Weight loss, smoking cessation, home oxygen

Erythrocytosis Key Points � Establish the cause

� Limited clinical guidelines for secondary erythrocytosis

� When in doubt – isovolaemic venesections

Extreme Cytosis Platelets � Definitions � Thrombocytosis > 400 x 109/l � Extreme thrombocytosis > 1000 x 109/l

� Reactive thrombocytosis

� Autonomous thrombocytosis � MPN, MDS, CML, AML

Extreme thrombocytosis Clinical presentation � Majority – Asymptomatic

� Vasomotor symptoms

� Thrombosis

� Bleeding

More common in Autonomous thrombocytosis

Extreme thrombocytosis Management � Bleeding

� Platelet dysfunction � Acquired type II VWD � Acquired Factor deficiencies (DIC, Factor V)

� Rx � Stop antiplatelet agents � Rapidly reduce the number of platelets � Platelet apheresis � Pharmacological agents – HU or Anagrelide

� Correct deficiencies

Extreme thrombocytosis Management � Thrombosis � Rx � Rapidly reduce the number of platelets � Platelet apheresis � Pharmacological agents – HU or Anagrelide

� Anticoagulate

� Vasomotor symptoms � Low dose Aspirin (81mg/day)

Thrombocytosis Key points � Level of thrombocytosis does NOT

differentiate causes

� Reactive causes – rarely complicates

� In case of bleeding / thrombosis – normalize platelet count urgently

Questions or Comments

Thank you