Leukaemia, Myeloma and Lymphoma

Report
Haematology revision
Shin Ying Lee
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.
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.
• What is the name of this complication of the
transfusion. 1
Acute hemolytic transfusion reaction
• 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.
• List 3 treatments that the patient might then
urgently require. 3
1. High flow oxygen
2. IV fluids
3. Diuretics
• 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
• What might you detect in the urine? 1
Hemoglobin
• 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)
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.
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.
• Name two investigations you would perform
and what are you likely to see? 2
1. Bloods – FBC, LFT. high reticulocytes count,
High bilirubin levels>50
2. Peripheral blood film- spherocytes,
elliptocytes
2. Urine sample – Urobilinogen in the urine
• 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
• She has a splenectomy. What two things
would you want to vaccinate against? 2
Pneumococcal vaccine
Haemophilus influenza type B vaccine
• 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, antimalarial precautions
• 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)
Leukaemia
• 4 types
Acute myeloid leukaemia (AML)
Acute lymphoid leukaemia (ALL)
Chronic myeloid leukaemia (CML)
Chronic lymphocytic leukaemia (CLL)
Investigations
• Blood count, White count differentials, red
cells, haemoglobin, plaletets
• Peripheral blood film
• Bone marrow biopsy and aspiration
• Immunophenotyping and molecular methods
• Cytogenetic analysis
CML
• Anaemia, bruising, unwell
• Dragging sensation at the abdomen/ abdomen
fullness (splenomegaly)
• Translocation of 9: 22. Philadelphia chromosome
>80% cases
• Chimeric gene (BCR/ABL) > phosphoprotein
(p190 and p210) with high tyrosine kinase
activity seen.
• spectrum of myeloid lineage cells
CML(peripheral blood film)
• Three phrases
- Chronic phase
- Accelerated phase
- Blast crisis
Treatment
• Imatinib (tyrosine kinase inhibitor)
Glivec/Gleevec
• Allogenic transplantation
CLL
• Swollen glands
• Constitutional signs and symptoms: Fever,
night sweats, weight loss
• Mature lymphocytes
• Transformed to Diffuse Large B cell lymphoma
Richter’s transformation
• Chemotherapy
• Stem cell transplant
CLL (peripheral blood film)
AML
• Neoplastic proliferation of blast cells derived
from myeloid elements.
• Bone marrow aspiration: auer rods, myeloid
precursors
AML
Clinical:
Good prognostic
factors
Bad prognostic
factors
Age
< 50 years
> 60 years
WBC count
<25,000/mm3
>25,000/mm3
FAB type
M3,M4eo
Mo
Auer rods
present
absent
Fibrosis
absent
absent
ALL
ALL
•
•
•
•
Affects children more commonly
Bleeding gums, recurrent infections, tiredness
Hepato-splenomegaly
Bone marrow aspiration: lymphoblast with
PAS positive
Treatment
Chemotherapy
• Remission induction : Vincristine,
prednisolone, L-asparaginase, daunorubicin
• Consolidation
• CNS prophylaxis: Intrathecal methotrexate+/CNS irradiation
• Maintenance
Bone marrow transplant
Prognostic factors
Clinical:
Good prognostic factors
Bad prognostic factors
age
4 to 10 years
> 10 years, or <2
years
WBC count
<50,000 mm3
>50,000 mm3
Immunophenotype
B-precursor ALL
Mature B or T cell
sex
girls
boys
Chromosomal number
hyperdiploidy
hypodiploidy
Lymphoma
• Two types:
Hodgkin’s 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-pelvis+BMA
• B symptoms: fever, weight loss, night sweats,
lethargy
Types
•
•
•
•
Nodular sclerosis (good prognosis)
Mixed cellularity
Lymphocyte rich
Lymphocyte depleted (poor)
Reed Sternberg Cell
• Nucleus is enclosed with an abundant
amphophilic cytoplasm, and contains large,
inclusion-like, owl eyed nucleoli, surrounded
by a clear halo.
• Hodgkin’s Lymphoma
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
Non Hodgkin’s Treatment
• Depending on subtype
• Low grade if symptomless none, localized
radiotherapy, diffuse (Chlorambucil)
• High grade (DLBCL) CHOP
• Cychophosphamide
• Hydroxydaunorubicin
• Oncovin (vincristine)
• Prednisolone
• +/- Rituximab
Myeloma
• Malignant clonal proliferation of B-lymphocyte
derived plasma cells
• Osteolytic bone lesions – unexplained
backache, pathological fractures
• Anaemic, neutropenia, thrombocytopenia
• Renal impairment
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
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
Treatment
• Supportive
• Chemotherapy
Complications of myeloma
•
•
•
•
Hypercalcaemia
Spinal cord compression
Hyperviscosity
Acute renal failure
4 causes of massive splenomegaly
•
•
•
•
CML
Malaria
Myelofibrosis
Thalassemia
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.
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.
Hodgkin’s lymphoma
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.
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
EMQ questions
• A 12-year-old girl of Nigerian descent and with
a known blood disorder presents to A&E 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.
EMQ questions
• A 12-year-old girl of Nigerian descent and with
a known blood disorder presents to A&E 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
EMQ questions
• A 65-year-old lady presents to her GP with a 3month 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.
EMQ questions
• A 65-year-old lady presents to her GP with a 3month 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
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
Schilling’s test.
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
Schilling’s test.
Pernicious anaemia
Thank you

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