Haematology revision

1
Haematology revision

• Shin Ying Lee

2
2010

• You are on cover for a surgical ward when you are
  called to Mrs McCulloch, who has just commenced a
  blood transfusion for a low post operative
  haemoglobin. Three minutes after the start of the
  transfusion the patient develops lumbar pains,
  rigors, dyspnoea and hypotension.

3
2010

• You are on cover for a surgical ward when you are
  called to Mrs McCulloch, who has just commenced a
  blood transfusion for a low post operative
  haemoglobin. Three minutes after the start of the
  transfusion the patient develops lumbar pains,
  rigors, dyspnoea and hypotension.

4

• What is the name of this complication of the
  transfusion. 1
• Acute hemolytic transfusion reaction

5

• What is the most likely procedural reason for
  this complication to have arisen? 1
• ABO incompatibility. Failure to check
  identity of patient when taking sample for
  compatibility testing, failure to perform paper
  identity checks before blood is transfused.

6

• List 3 treatments that the patient might then
  urgently require. 3
• High flow oxygen
• IV fluids
• Diuretics

7

• Name 2 further steps that would need to be taken
  rapidly. 1
• 1. Send donor blood back to blood bank with
  notification of event
• 2. Inform hospital transfusion department
  immediately

8

• What might you detect in the urine? 1
• Hemoglobin

9

• List three further short term complications of
  blood transfusions. 3
• 1. Febrile non-hemolytic reaction
• 2. Allergic and anaphylactic reaction
• 3. TRALI (Transfusion related acute lung
  injury)

10
2007

• A 40 year old woman presents with recent
  tiredness and lethargy. She has pale conjunctivae
  and you think there may be some yellowing of the
  sclera.

11
2007

• A 40 year old woman presents with recent
  tiredness and lethargy. She has pale conjunctivae
  and you think there may be some yellowing of the
  sclera.

12

• Name two investigations you would perform and
  what are you likely to see? 2
• 1. Bloods FBC, LFT. high reticulocytes
  count, High bilirubin levelsgt50
• 2. Peripheral blood film- spherocytes,
  elliptocytes
• 2. Urine sample Urobilinogen in the urine

13

• What two abnormalities might you expect to see on
  biochemistry and haematology in haemolytic
  anaemia? 2
• Biochem- High bilirubin levels, high LDH
• Haemato- Low hemoglobin, increase
  reticulocytes count

14

• She has a splenectomy. What two things would you
  want to vaccinate against? 2
• Pneumococcal vaccine
• Haemophilus influenza type B vaccine

15

• What two pieces of advice would you want to give
  her in regards to her asplenism?2
• 1) Lifelong prophylactic antibiotics
• 2) Reimmunisation every 5 years, annual
  influenza vaccine
• 3) Increased risk of malaria falciparum,
  anti-malarial precautions

16

• Name two causes of haemolytic anaemia. 2
• 1) Abnormal membrane (Hereditary
  spherocytosis, elliptocytosis)
• 2) Abnormal enzymes (G6PD deficiency)
• 3) Abnormal hemoglobin synthesis
  (thalassemia, hemoglobinopathies, sickle cell
  disease)

17
Leukaemia

• 4 types
• Acute myeloid leukaemia (AML)
• Acute lymphoid leukaemia (ALL)
• Chronic myeloid leukaemia (CML)
• Chronic lymphocytic leukaemia (CLL)

18
Investigations

• Blood count, White count differentials, red
  cells, haemoglobin, plaletets
• Peripheral blood film
• Bone marrow biopsy and aspiration
• Immunophenotyping and molecular methods
• Cytogenetic analysis

19
CML

• Anaemia, bruising, unwell
• Dragging sensation at the abdomen/ abdomen
  fullness (splenomegaly)
• Translocation of 9 22. Philadelphia chromosome
  gt80 cases
• Chimeric gene (BCR/ABL) gt phosphoprotein (p190
  and p210) with high tyrosine kinase activity
  seen.
• spectrum of myeloid lineage cells

20
CML(peripheral blood film)
21

• Three phrases
• - Chronic phase
• - Accelerated phase
• - Blast crisis

22
Treatment

• Imatinib (tyrosine kinase inhibitor)
  Glivec/Gleevec
• Allogenic transplantation

23
CLL

• Swollen glands
• Constitutional signs and symptoms Fever, night
  sweats, weight loss
• Mature lymphocytes
• Transformed to Diffuse Large B cell lymphoma
• Richters transformation
• Chemotherapy
• Stem cell transplant

24
CLL (peripheral blood film)
25
AML

• Neoplastic proliferation of blast cells derived
  from myeloid elements.
• Bone marrow aspiration auer rods, myeloid
  precursors

26
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27
AML
Clinical Good prognostic factors Bad prognostic factors
Age lt 50 years gt 60 years
WBC count lt25,000/mm3 gt25,000/mm3
FAB type M3,M4eo Mo
Auer rods present absent
Fibrosis absent absent
28
ALL
29
ALL

• Affects children more commonly
• Bleeding gums, recurrent infections, tiredness
• Hepato-splenomegaly
• Bone marrow aspiration lymphoblast with PAS
  positive

30
Treatment

• Chemotherapy
• Remission induction Vincristine, prednisolone,
  L-asparaginase, daunorubicin
• Consolidation
• CNS prophylaxis Intrathecal methotrexate/- CNS
  irradiation
• Maintenance
• Bone marrow transplant

31
Prognostic factors
Clinical Good prognostic factors Bad prognostic factors
age 4 to 10 years gt 10 years, or lt2 years
WBC count lt50,000 mm3 gt50,000 mm3
Immunophenotype B-precursor ALL Mature B or T cell
sex girls boys
Chromosomal number hyperdiploidy hypodiploidy
32
Lymphoma

• Two types
• Hodgkins and Non Hodgkins
• Malignancy of the lymph nodes
• Diagnosis confirmation lymph node biopsy
• Bloods plus LDH, uric acid
• CXR bihilar lymphadenopathy
• Staging CT scan chest, abdo-pelvisBMA
• B symptoms fever, weight loss, night sweats,
  lethargy

33
Types

• Nodular sclerosis (good prognosis)
• Mixed cellularity
• Lymphocyte rich
• Lymphocyte depleted (poor)

34
Reed Sternberg Cell

• Nucleus is enclosed with an abundant amphophilic
  cytoplasm, and contains large, inclusion-like,
  owl eyed nucleoli, surrounded by a clear halo.
• Hodgkins Lymphoma

35
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36
Ann Arbor staging

• Stage I - a single lymph node area or single
  extranodal site
• Stage II - 2 or more lymph node areas on the same
  side of the diaphragm
• Stage III - denotes lymph node areas on both
  sides of the diaphragm
• Stage IV - disseminated or multiple involvement
  of the extranodal organs

37
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38
Non Hodgkins Treatment

• Depending on subtype
• Low grade if symptomless none, localized
  radiotherapy, diffuse (Chlorambucil)
• High grade (DLBCL) CHOP
• Cychophosphamide
• Hydroxydaunorubicin
• Oncovin (vincristine)
• Prednisolone
• /- Rituximab

39
Myeloma

• Malignant clonal proliferation of B-lymphocyte
  derived plasma cells
• Osteolytic bone lesions unexplained backache,
  pathological fractures
• Anaemic, neutropenia, thrombocytopenia
• Renal impairment

40
Investigations

• Bloods Blood count, Hypercalcemia- increase
  osteoclastic activity
• Serum and urine electrophoresis
• Urine sample- Bence Jones protein K, Lambda
  light chains
• B2 microglobulin prognostic test
• Skeletal survey- Lytic punched out lesion

41
Diagnostic criteria

• Monoclonal protein band in serum or urine
  electrophoresis
• Increased plasma cells found on BMB
• Evidence of end organ damage from myeloma
• - Hypercalcemia
• - Renal insufficiency
• - Anaemia
• - Bone lesions

42
Treatment

• Supportive
• Chemotherapy

43
Complications of myeloma

• Hypercalcaemia
• Spinal cord compression
• Hyperviscosity
• Acute renal failure

44
4 causes of massive splenomegaly

• CML
• Malaria
• Myelofibrosis
• Thalassemia

45
EMQ questions

• A 27-year-old man presents with a two-month
  history of pruritis, fatigue and weight loss. On
  questioning he admits that whenever he drinks
  alcohol, he experiences bone pain. On examination
  he has a rubbery non-tender submandibular lymph
  node. He has never had infectious mononucleosis.

46
EMQ questions

• A 27-year-old man presents with a two-month
  history of pruritis, fatigue and weight loss. On
  questioning he admits that whenever he drinks
  alcohol, he experiences bone pain. On examination
  he has a rubbery non-tender submandibular lymph
  node. He has never had infectious mononucleosis.
• Hodgkins lymphoma

47
EMQ questions

• A 68-year-old woman presents with a history of
  bruising, bone pain and lymphadenopathy.
  Unbeknownst to the consultant, this patient has a
  (t9,22) mutation known as the Philadelphia
  Chromosome. On examination the consultant finds a
  massively enlarged spleen.

48
EMQ questions

• A 68-year-old woman presents with a history of
  bruising, bone pain and lymphadenopathy.
  Unbeknownst to the consultant, this patient has a
  (t9,22) mutation known as the Philadelphia
  Chromosome. On examination the consultant finds a
  massively enlarged spleen.
• Chronic Myeloid Leukaemia

49
EMQ questions

• A 12-year-old girl of Nigerian descent and with a
  known blood disorder presents to AE with a
  two-day history of dyspnoea, cough and fever. You
  order several investigations and note that she
  has a Hb of 6g/dl (reference range 11.5 1.35
  g/dl) and a chest X-ray showing pulmonary
  infiltrates.

50
EMQ questions

• A 12-year-old girl of Nigerian descent and with a
  known blood disorder presents to AE with a
  two-day history of dyspnoea, cough and fever. You
  order several investigations and note that she
  has a Hb of 6g/dl (reference range 11.5 1.35
  g/dl) and a chest X-ray showing pulmonary
  infiltrates.
• Sickle cell anaemia

51
EMQ questions

• A 65-year-old lady presents to her GP with a
  3-month history of vertigo, tinnitus and visual
  disturbance. She admits to feeling a bit down
  and the GP decides to carry out some routine
  bloods. A week later she returns and you note
  that her blood results show a raised high
  haemoglobin and a raised pack cell volume and red
  blood cell count.

52
EMQ questions

• A 65-year-old lady presents to her GP with a
  3-month history of vertigo, tinnitus and visual
  disturbance. She admits to feeling a bit down
  and the GP decides to carry out some routine
  bloods. A week later she returns and you note
  that her blood results show a raised high
  haemoglobin and a raised pack cell volume and red
  blood cell count.
• Polycythaemia vera

53
EMQ questions

• A 37 year old lady with known hypothyroidism
  presents to you with fatigue, dyspnoea and
  palpitations. You note that she is pale and
  tachycardic. Routine bloods show a macrocytic
  anaemia. You suspect that this is caused by her
  hypothyroidism. You find a positive Schillings
  test.

54
EMQ questions

• A 37 year old lady with known hypothyroidism
  presents to you with fatigue, dyspnoea and
  palpitations. You note that she is pale and
  tachycardic. Routine bloods show a macrocytic
  anaemia. You suspect that this is caused by her
  hypothyroidism. You find a positive Schillings
  test.
• Pernicious anaemia

55

• Thank you