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Haematological emergencies
Dr Rajesh Krishna
MD ( Gen Med), MRCP(UK), FRCPath, CCT ( Haem )
Consultant Haematologist
Case-1
• 53 year old lady
• No significant past history
• Fatigue
• Extensive bruising
• Fever
• Hb 7.2 g/dL
• WBC 3.2 x 10*9/L
• Plts 12 x 10*9/L
• PT 36 sec
• APTT 52 sec
• Creatinine- 0.9 mg/dL
APML
APML
• ATRA
• Platelet transfusion
• Correct clotting
• Fluid and electrolyte management
• Management of infection
• Bone marrow biopsy
• Next day : develops Fever
Neutropenic sepsis
• Fever > 38 C in neutropenic patient
• Medical emergency
• Needle to patient < 30 minutes
• Hydration
• Blood cultures
• Broad spectrum antibiotics
• CXR, urine culture
Central line sepsis
• Check lines
• Line cultures
• Severe sepsis, hypotensive patient
• Multi drug resistant organsisms
• Line out, tip cultures
Treatment of APML
• t(15:17): PML-RARA
• Good risk disease
• High risk of DIC in the first 2 weeks of
treatment ( activates Tissue factor )
• All trans retinoic acid promotes maturation of
pro myelocytes
• Also given Arsenic trioxide
• High risk disease given chemotherapy
6 days later
ATRA Differentiation syndrome
Case-2
• 18 year old girl
• Fainting episode
• Bruising
• Hb 7.2 g/dL
• WCC 320 x 10*9/L
• Plts 24 x 10*9/L
• Precursor B- Acute lymphoblastic leukaemia
• Steroid pre phase
• Next day
• K- 5.5 mmol/L
• Creatinine 1.6 mg/dL
Tumour lysis syndrome
• Bulky disease/ High WCC
• Raised LDH
• Hyper uricemia, hyperphosphatemia,
• Hypocalcemia
• Raised creatinine, hyperkalemia
• Aggressive hydration
• Allopurinol : inhibits xanthine oxidase
• Rasburicase : urate oxidase
Case-3
• 59 year old man
• Shortness of breath
• Fatigue
• Headache
• Occasional visual blurring
• Back pain
• Hb 9.8 g/dL
• WCC 5.6 x 10*9/L
• Plts 98 x 10*9/L
• Creatinine 2.0 mg/dL
• Electrolytes normal
• Raised globulins
Serum protein electrophoresis/
Urine bence jones protein
Serum free light chains
Serum protein electrophoresis and
serum free light chains
• IgG 93 g/L (6-16)
• IgA 0.1 g/L ( 0.8-4)
• IgM 0.2 g/L ( 0.5-2)
• Paraprotein 62 g/L
• SFLC kappa 12 mg/L(3-19)
• SFLC Lambda 1369 mg/L( 5-27)
• SFLC Ratio 0.01 (0.26-1.65)
Skeletal survey
• Lytic lesions
• Fractures
Bone marrow
aspirate/ Trephine
biopsy
• Plasma cells > 10 %
• They crowd out
normal bone marrow
architecture
Renal Biopsy
• Cast nephropathy
Diagnosis = Myeloma if following
criteria met
• Paraprotein in blood/ urine/Abnormal serum free light chain
ratio
• Bone marrow plasma cells > 10 %
• CRAB ( Hypercalcemia/ Renal impairment/ Anaemia/ Bony
lytic lesions)
MGUS ( Monoclonal gammapathy of
undetermined significance ) if
• Paraprotein in blood/ urine/Abnormal serum free light chain
ratio
• Bone marrow plasma cells less than 10 %
• Negative for CRAB ( Hypercalcemia/ Renal impairment/
Anaemia/ Bony lytic lesions)
Diagnosis = Asymptomatic Myeloma
• Paraprotein in blood/ urine/Abnormal serum free light chain
ratio
• Bone marrow plasma cells > 10 %
• Negative for CRAB ( Hypercalcemia/ Renal impairment/
Anaemia/ Bony lytic lesions)
Diagnosis = Myeloma if following
criteria met
• Paraprotein in blood/ urine/Abnormal serum free light chain
ratio
• Bone marrow plasma cells > 10 %
• CRAB ( Hypercalcemia/ Renal impairment/ Anaemia/ Bony
lytic lesions)
Hyper viscosity: Causes
• Acute leukaemia with high counts
• Waldenstrom macroglobinaemia
• Multiple myeloma
• Sickle cell anaemia
• CML with high counts
Hyperviscosity: Treatment
• Hydration
• Plasmapheresis ( plasma exchange)
• DVT prophylaxis
• Management of cause
• Fluid and electrolyte management
Cord compression
• Weakness of legs
• Bladder, bowel dysfunction
• Emergency management
• MRI spine
• Steroids : High dose dexamethasone
• Neurosurgery opinion
• Radiotherapy
• Definitive management
1st line
treatment
2nd line
treatment
3rd line
treatment
MGUS: Monoclonal gammopathy of
undetermined significance
Natural history of myeloma
Intensive and non-intensive Options
Are you a candidate for
stem cell transplant
Symptomatic
myeloma
Clinical
trial
Yes
Induction treatment,
stem cell transplant
No
Non-intensive
drug treatment
Initial treatment
Thalidomide Velcade Revlimid
CTD
TD
PAD
VCD
VTD
VRD
VTD Dara
RCD
Thalidomide Velcade Doublets
MPT
CTD
VMP
VCD
MP
CD
TD
VD
Intensive
(induction prior
to transplant)
Non-intensive
Transfusion associated Graft versus
host disease
• Post bone marrow transplant
• Severe T cell immunosuppression
• skin rash, liver dysfunction, diarrhoea
• Invariably fatal
• Prevention: Irradiated blood products and
leukodepletion
Case-4
• 28 year old lady
• Bruises
• Generally unwell
• Anaemia
• Platelets 30 x 10*9/L
• Headaches
TTP
• Thrombocytopenia
• MAHA
• Fever
• Renal failure
• Neurology
• Haematological emergency
• ADAMTS13 deficiency ( VWF cleaving protein)
• Large VWF multimers cause plt aggregates in
microvasculature
• Plasma exchange 1-5 volumes x 3 days
• Steroids and iv methyl pred
• Folic acid
• Aspirin and LMWH when Plts>50
DD
• HUS : history of diarrhoea and oliguric/ anuric
renal failure
• DIC : Abnormal clotting
Thank You

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Haematological emergencies

  • 1. Haematological emergencies Dr Rajesh Krishna MD ( Gen Med), MRCP(UK), FRCPath, CCT ( Haem ) Consultant Haematologist
  • 2. Case-1 • 53 year old lady • No significant past history • Fatigue • Extensive bruising • Fever
  • 3. • Hb 7.2 g/dL • WBC 3.2 x 10*9/L • Plts 12 x 10*9/L • PT 36 sec • APTT 52 sec • Creatinine- 0.9 mg/dL
  • 5. APML • ATRA • Platelet transfusion • Correct clotting • Fluid and electrolyte management • Management of infection • Bone marrow biopsy • Next day : develops Fever
  • 6. Neutropenic sepsis • Fever > 38 C in neutropenic patient • Medical emergency • Needle to patient < 30 minutes • Hydration • Blood cultures • Broad spectrum antibiotics • CXR, urine culture
  • 7. Central line sepsis • Check lines • Line cultures • Severe sepsis, hypotensive patient • Multi drug resistant organsisms • Line out, tip cultures
  • 8. Treatment of APML • t(15:17): PML-RARA • Good risk disease • High risk of DIC in the first 2 weeks of treatment ( activates Tissue factor ) • All trans retinoic acid promotes maturation of pro myelocytes • Also given Arsenic trioxide • High risk disease given chemotherapy
  • 11. Case-2 • 18 year old girl • Fainting episode • Bruising • Hb 7.2 g/dL • WCC 320 x 10*9/L • Plts 24 x 10*9/L
  • 12.
  • 13. • Precursor B- Acute lymphoblastic leukaemia • Steroid pre phase • Next day • K- 5.5 mmol/L • Creatinine 1.6 mg/dL
  • 14. Tumour lysis syndrome • Bulky disease/ High WCC • Raised LDH • Hyper uricemia, hyperphosphatemia, • Hypocalcemia • Raised creatinine, hyperkalemia • Aggressive hydration • Allopurinol : inhibits xanthine oxidase • Rasburicase : urate oxidase
  • 15. Case-3 • 59 year old man • Shortness of breath • Fatigue • Headache • Occasional visual blurring • Back pain
  • 16. • Hb 9.8 g/dL • WCC 5.6 x 10*9/L • Plts 98 x 10*9/L • Creatinine 2.0 mg/dL • Electrolytes normal • Raised globulins
  • 19. Serum protein electrophoresis and serum free light chains • IgG 93 g/L (6-16) • IgA 0.1 g/L ( 0.8-4) • IgM 0.2 g/L ( 0.5-2) • Paraprotein 62 g/L • SFLC kappa 12 mg/L(3-19) • SFLC Lambda 1369 mg/L( 5-27) • SFLC Ratio 0.01 (0.26-1.65)
  • 20. Skeletal survey • Lytic lesions • Fractures
  • 21. Bone marrow aspirate/ Trephine biopsy • Plasma cells > 10 % • They crowd out normal bone marrow architecture Renal Biopsy • Cast nephropathy
  • 22. Diagnosis = Myeloma if following criteria met • Paraprotein in blood/ urine/Abnormal serum free light chain ratio • Bone marrow plasma cells > 10 % • CRAB ( Hypercalcemia/ Renal impairment/ Anaemia/ Bony lytic lesions)
  • 23. MGUS ( Monoclonal gammapathy of undetermined significance ) if • Paraprotein in blood/ urine/Abnormal serum free light chain ratio • Bone marrow plasma cells less than 10 % • Negative for CRAB ( Hypercalcemia/ Renal impairment/ Anaemia/ Bony lytic lesions)
  • 24. Diagnosis = Asymptomatic Myeloma • Paraprotein in blood/ urine/Abnormal serum free light chain ratio • Bone marrow plasma cells > 10 % • Negative for CRAB ( Hypercalcemia/ Renal impairment/ Anaemia/ Bony lytic lesions)
  • 25. Diagnosis = Myeloma if following criteria met • Paraprotein in blood/ urine/Abnormal serum free light chain ratio • Bone marrow plasma cells > 10 % • CRAB ( Hypercalcemia/ Renal impairment/ Anaemia/ Bony lytic lesions)
  • 26. Hyper viscosity: Causes • Acute leukaemia with high counts • Waldenstrom macroglobinaemia • Multiple myeloma • Sickle cell anaemia • CML with high counts
  • 27. Hyperviscosity: Treatment • Hydration • Plasmapheresis ( plasma exchange) • DVT prophylaxis • Management of cause • Fluid and electrolyte management
  • 28.
  • 29. Cord compression • Weakness of legs • Bladder, bowel dysfunction • Emergency management • MRI spine • Steroids : High dose dexamethasone • Neurosurgery opinion • Radiotherapy • Definitive management
  • 30. 1st line treatment 2nd line treatment 3rd line treatment MGUS: Monoclonal gammopathy of undetermined significance Natural history of myeloma
  • 31. Intensive and non-intensive Options Are you a candidate for stem cell transplant Symptomatic myeloma Clinical trial Yes Induction treatment, stem cell transplant No Non-intensive drug treatment
  • 32. Initial treatment Thalidomide Velcade Revlimid CTD TD PAD VCD VTD VRD VTD Dara RCD Thalidomide Velcade Doublets MPT CTD VMP VCD MP CD TD VD Intensive (induction prior to transplant) Non-intensive
  • 33. Transfusion associated Graft versus host disease • Post bone marrow transplant • Severe T cell immunosuppression • skin rash, liver dysfunction, diarrhoea • Invariably fatal • Prevention: Irradiated blood products and leukodepletion
  • 34. Case-4 • 28 year old lady • Bruises • Generally unwell • Anaemia • Platelets 30 x 10*9/L • Headaches
  • 35.
  • 36. TTP • Thrombocytopenia • MAHA • Fever • Renal failure • Neurology
  • 37. • Haematological emergency • ADAMTS13 deficiency ( VWF cleaving protein) • Large VWF multimers cause plt aggregates in microvasculature • Plasma exchange 1-5 volumes x 3 days • Steroids and iv methyl pred • Folic acid • Aspirin and LMWH when Plts>50
  • 38. DD • HUS : history of diarrhoea and oliguric/ anuric renal failure • DIC : Abnormal clotting

Editor's Notes

  1. 30