Types of leukaemia Flashcards
Classification of acute leukaemia based on..
Morphology Cytochemistry Immunophenotype (flow cytometry) Genetic abnormalities (karyotype,FISH,PCR)
Define acute leukaemia
Presence of at least 20% blasts in the PB or BM
What is most common malignancy in children
ALL
Most common secondary leukaemia
AML
Morphology in AML
Large blasts
Primitive nuclei
Granules
Auer rods
AML cytochemistry
Sudan black B pos
MPO pos
AML flow cytometry
CD13
CD33
Cytogenetics of AML
t(8:21)
t(15:17)
inv(16)
Morphology ALL
Scanty cytoplasm
Primitive nuclei
No granules
No auer rods
Cytochemistry of ALL
Sudan black neg
MPO neg
Flow cytometry ALL
B - CD10, CD 19
T - CD7, CD3
Cytogenetics ALL
t(4:11)
t(12:21)
Markers for immature cells
CD34
HLA-DR
Myeloid marker
CD13
T cell marker
CD 2
B cell markers
CD 19, CD 10
How to prevent infections
Single room (reverse isolation) Regular hand washing Avoid contact with any sick person No fresh fruit or raw veg Antibiotics and anti fungal prophylaxis Careful oral care Care of IV lines
Translocations of Burkitt’s
t(8:14)
t(2:8)
t(8:22)
8=myc oncogene
14,2,22 = immunoglobulin chain
Types of Burkitt’s
Endemic - jaw mass
Sporadic - abdo mass, ascites
Immunodeficiency associated - advanced
Egs of chronic myeloproliferative disorders
CML
Polycythemia Vera
Essential Thrombocytopenia
Primary myelofibrosis
Cytogenetic finding in CML
t(9:22) Philadelphia chr
9 - BCR/ABL. 22 - light chain
What does increase BCR/ABL cause
Increase tyrosine kinase activity
Increased cell proliferation
Decreased apoptosis
Adherence of BM cells to stroma
Course of CML
- Initial chronic stable phase
- Accelerated phase
- Blast phase
Rx of CML
Tyrosine kinase inhibitors (not cure) BM transplant (cure)
Underlying pathology on BCR/ABL neg
JAX2 mutation = unregulated proliferation of granulocytes, megakaryocytes and erythrocytes
Causes of increased RBC other than PV
1ry familial polycythemia
Ectopic EPO production
Chronic tissue hypoxia
Relative - dehydration, burns
Lab findings in PV
Increase:
RBC, Hb, hematocrit, platelet, blood viscosity
Abnormal megs - hypersegmented nuclei
Rx of PV
Repeated venesection
Long term warfarin
Aspirin
Cytoreductive agents
Course of PV
Prodromal, prepolycythemic phase
Overt polycythemic phase
Spent/ postpolycythemic phase
Reactive causes of thrombocytosis
- Infection/inflammation
- Chronic haemorrhage
- Fe def
- Post surgery
- Malignancy
Why can ET present with bleeding despite increased platelets
Platelets are mostly non functional
Rx of ET
Control platelet count
Cytoreductive agents
Aspirin
Define primary myelofibrosis
Progressive generalized reactive fibrosis of BM with haemopoiesis in liver and spleen. BM fibrosis due to megakaryocytes proliferation -> PDGF.
Course of primary myelofibrosis
Prefibrotic phase
Fibrotic phase
Rx of primary myelofibrosis
Palliative
Reduce anaemia HSM
In kids BM transplant may cure
Chronic B cell leukaemic types
CLL
Hairy cell leukaemia
B prolymphocytic leukaemia
Chronic T cell leukaemic types
T prolymphocytic leukaemia
Large granular lymphocytic leukaemia
Adult T leukaemia/Lymphoma
Presentation of hairy cell leukaemia
Severe infection
Anaemia
Splenomegaly
Clinical picture with multiple myeloma
BM infiltration - bone pain, fractures, amaemia, infection, bleeding, osteolytic lesions -> renal calcification
Monoclonal proteins - renal failure (high calcium, urea, light chains) hyperviscosity, peripheral neuropathy, amyloidosis, high ESR
Diagnosis of multiple myeloma
Monoclonal Ig Immunotyping Hence Jones proteins in urine Many plasma cells in aspirate (X-ray, ESR) NB exclude other causes of monoclonal Ig
Define amyloidosis
Deposition of protein extracellularly that disrupts tissue/organ function.
Organs affected by systemic amyloidosis that generally show clinically
Heart - failure
Nerves - neuropathy, carpal tunnel
Kidneys - failure
Tongue - macroglossia
