Lymphoid and myeloid disorders Flashcards
What is acute leukaemia?
- Proliferation of primitive precursor cells usually only found in bone marrow.
- Proliferation without
- Replaces normal bone marrow cells, leads to:
- Anaemia: Palor and lethargy
- Neutropenia: Infections
- Thrombocytopenia: bleeding
Bone pain due to marrow infiltration
How do we classify ACUTE Lymphoid and Myeloid Leukaemia’s
Acute & Lymphoid Leukaemia = ACUTE LYMPHOBLASTIC LEUKAEMIA
Acute & Myeloid = ACUTE MYELOID LEUKAEMIA
How do we classify CHRONIC Lymphoid and Myeloid Leukaemia’s
Chronic & Lymphoid Leukaemia = CHRONIC LYMPHATIC LEUKAEMIA
Chronic & Myeloid = CHRONIC MYELOID LEUKAEMIA
What is acute lymphoblastic leukaemia
- Malignant proliferation of lymphoblasts in bone marrow
- Affects mainly children
- Good prognosis - 85% cure rate (esp girls age 1-10). Poor prognosis in adults
How do we treat Acute lymphoblastic leukaemia
- Induction chemotherapy
- Consolidation chemotherapy +/- craniospinal irradiation
- Maintenance chemotherapy
- Bone marrow transplantation only if relapse
What is acute myeloid leukaemia
- Malignant proliferation of myeloblasts in bone marrow
- Affects mostly adults. Poor prognosis 15-50% 5 year survival (depends on subtype). MOST patients relapse
- Gum infiltration in acute monocytic subtype (M5)
How do we treat acute myeloid leukaemia?
- Cyclic high dose chemotherapy (induction and consolidation with NO maintenance)
- Sometimes bone marrow transplantation
What is Chronic lymphatic leukaemia?
- Proliferation of mature lymphocytes, usually B cells
- Commonest leukaemia
- Affects elderly >65 years
- Presents with anemia, infections, lymphadenopathy, splenomegaly
- Lymphocytosis on blood film
- Good prognosis
- Survival >10 years is the norm. Treat only in advanced disease
What is Chronic myeloid leukaemia
- Gradual onset
- High white cell count & splenomegaly
- Philadelphia chromosome (t9;22) (translation between chromosome 9 and 22)
- BCR-ABL tyrosine kinase (2 oncogenes with translocation)
- 3 phases: chronic, accelerated and blast crisis
How do we treat chronic myeloid leukaemia?
• Imatinib (Glivec)
- Blocks abnormal BCR-ABL tyrosine kinase activity
- Can result in molecular remission
• Allogeneic stem cell transplantation
What is myelodysplasia
- Premalignant condition of haemopoietic precursors
- A disease of the elderly. Can be ASYMPTOMATIC
- May be present with anaemia, thrombocytopenia, pancytopenia
- Several subtypes. Variable course
- Can transform to acute myeloid leukaemia
- Treat with supportive care and bone marrow transplantation in the young.
What are the two types of lymphoma?
- ) Hodgkin lymphoma
2. ) Non-hodgkin lymphoma
Describe Hodgkin lymphoma
- Painless lymphadenopathy
- B symptoms (advanced): sweats, weight loss, fever
- 4 subtypes
- Reed-Sternberg cell diagnostic on biopsy
- Treatment:
- Chemotherapy
- Radiotherapy
- Stem cell transplantation
Describe Low grade Non Hodgkin lymphoma
- Gradual onset
- May be asymptomatic
- Incurable (10yrs survival)
• Treatment: Chemotherapy, Radiotherapy, Transplantation
Describe High grade Non-Hodgkin lymphoma
- Rapidly progressive
- Usually symptomatic
- Potentially curable
- Treatment: Chemotherapy, radiotherapy, transplantation
What is the disorder involving uncontrolled red cell proliferation
• Polycythaemia Rubra Vera (PRV)
What is the disorder involving white cell proliferation
Chronic Myeloid Leukaemia
What is the disorder involving platelet proliferation
Essential Thrombocythaemia
What is marrow stroma proliferation
Myelofibrosis
What is another name for polycythaemia? Describe the two main ways of acquiring it
Erythocytosis
- ) PRIMARY:
- Polycythaemia rubra vera
- Pruritus, plethoric facies
- Thrombosis
- Splenomegaly, hepatomegaly
- Treatment: Venesection, aspirin, myelosuppression
- Outcome: Myelofibrosis (15-20%), AML (2-10%)
- ) SECONDARY:
- Hypoxic: High altitude, lung or cyanotic heart disease
- Inappropriate erythropoietin secretion: renal tumour.
What is thrombocytosis
Primary: Essential thrombocythaemia
- Uncontrolled malignant proliferation of megakaryocytes
- Platelets >600 persistently
- Arterial and venous thrombosis
- Bleeding with very high counts e.g. >1500
- Treatment with aspirin, hydroxycarbamide, anagrelide, interferon
Secondary:
- Bleeding, infection, inflammation, malignancy
What is multiple myeloma
- Malignant proliferation of plasma cells (terminally differentiated B cells that produce immunoglobulin) in the bone marrow
- Myeloma has monoclonal immunoglobulin in blood and urine
Symptoms of myeloma
• Lytic lesions in bones
- pain and pathological fractures
• Hypercalcaemia due to bone resorption
- thirst, pylori, confusion, constipation
- Hyperviscocity (thick blood) due to immunoglobulin
- Renal failure
- Anaemia
- Infections
How do we diagnose and treat myeloma?
DIAGNOSIS:
- Blood and urine tests
- x-rays and CT/MRI
- Bone marrow
TREATMENT:
- Chemotherapy
- Thalidomide
- Radiation
- Stem cell transplantation