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Haematological Malignancy (Myeloma (What is it? Malignant proliferation…
Haematological Malignancy
Leukaemia
Acute
Acute Lymphoblastic Leukaemia
What is it?
malignant transformation of lymphoid progenitor cells
Cells of bone marrow replaced
Cells spill out into blood
Who gets it?
Any age
80% of childhood leukaemias are ALL
Symptoms
Fatigue, dizziness, palpitations
Pallor
Unusual joint or bone pain
Infection
-Splenomegaly
Investigations
FBC - low HB, low platelets
blood film - blasts
genetic studies
Acute Myeloid Leukaemia
What is it?
Malignant disease of bone marrow
Arrest and accumulation of myeloid progenitor cells
Who gets it?
Incidence increases with age
Most common adult leukaemia
Signs/ Symptoms
Neutropnenia
Anaemia
Thombocytopenia
Organ failure
Investigations
FBC - Low, HB, low platelets, low neutrophils
Bone marrow aspiration (diagnostic test)
Cytogentic studies (prognostic test)
Chronic
Chronic Lymphocyitc Leukaemia
What is it?
malignant monoclonal expansion of B lymphocytes
Accumulation of blood, marrow, nodes and spleen
Who gets it?
More common in elderly (mean age 72)
Most common leukaemia in western world
Signs/ symptoms
Insidious onset
Enlarged lymph nodes
Infection
Splenomegaly
Investigation
FBC - raised lymphocytes
Blood film
Chronic myeloid Leukaemia
What is it?
myeloproliferative disorder of myeloid progenitor cells
Has chronic and accelerated phases
90% hay Philadelphia mutation
BRC-ABL tryrosine kinase constitutively activated
Who gets it?
Any age
Rare in children
Mean age in 60-65
Signs / Symptoms
Often incidental
Fatigue
Night sweats
Massive Splenomegaly
Investigations
FBC - leukocytosis
Blood film
Bone marrow aspiration
Lymphoma
non - Hodgkin Lymphoma
What is it?
Lymphoproliferative disease
Majority a malignancy of B cells
Divided into low grade and high grade
Who gets it?
Elderly
Low grade very rare in children
Signs/ symptoms
Low grade
lymphadenopathy
Splenomegaly
High Grade
Rapid growing lymphadenopathy
Hepatosplenomegaly
Investigations
FBC
Biopsy (preferably by excision)
CT (look for nodes)
Hodgkin Lymphoma
What is it?
Malignancy of the lymphatic system
Reed sternberg cells present
Malignancy of B-cells
Who gets it?
Any age
Slightly more common in men
Signs / symptoms
Enlarged lymph nodes
Mediastinal masses
Drenching night sweats
Investigations
FBC
Biopsy
HIV test
Myeloma
What is it?
Malignant proliferation of plasma cells
Over production of a monoclonol antibody
IgG Antibodies most common
Who gets it?
Elderly
More common in Afroamerican
More common in men
Signs /symptoms
Bone pain
Pathological fractures
Spinal cord compression
Lethargy
Investigation
Urine protein - bence jones protein
Serum electrophoresis - looking for paraprotein
Bone marrow aspirate with plasma cell phenotyping
Myeloid proliferative disease
Myelofibrosis
What is it?
Clonal haematopoetic stem cell proliferation
Marrow cells in blood, liver, spleen
Who gets it?
More common in elderly
Slightly more common in white people and men
Signs/ symptoms
Variable presentation
Massive splenomegaly
Anaemia
Investigation
FBC
Blood film
Bone marrow aspirate
Cytogenetic testing
Chronic Myeloid Leukaemia
Polycythaemia Rubra vera
What is it?
Clonal haematological malignancy
Jak 2 mutation in 90% cases
Over production of primarily RBCs
Who gets it?
Elderly
Signs / symptoms
Incidental finding
Thrombosis
headache, dizziness, sweating
Investigation
FBC - raised HB, platelets and WCC
Low ferratin
Jak 2 testing
Essential Thrombocythaemia
What is it?
Malignant proliferation of Megakaryocytes
JAK 2 mutation often present
Who gets it?
Median age is 60
Usually chronic
Signs / symptoms
Vascular occlusion
incidental finding
Investigations
FBC - isolated raised platelets