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Lymphoproliferative Disorders - Coggle Diagram
Lymphoproliferative Disorders
Acute Lymphocytic Leukaemia (ALL)
Risk Factors
T-ALL = peak incidence at 15-20 yrs old
Down syndrome = after age 5
B-ALL: peak incidence at 3 yrs old
Radiation exposure
Young age (common leukaemia in children)
Complications
Secondary malignancies
Congenital problems in offspring of survivors
Type
B Cell ALL
85% of cases
Origin = Pre-B cells of bone marrow
T Cell ALL
Origin = Pre-T cells in thymus
Pathology
Neoplastic monoclonal proliferation of lymphoid stem cells (lymphoblasts) in BM
Onset = rapid
Immature lymphoblasts accumulate in BM → crowding and suppress hematopoiesis → prevent maturation → pancytopaenia
Others
Prognosis
Child and adult patients = risk of developing CNS involvement during course of the disease → treatment and prognosis are influenced by this complication
Chromosomal abnormalities - Ph (+) ALL
CNS involvement
↓ prognosis and represent > than 30% of adult cases
Better prognosis in patients < 25 years; better prognosis in patients < 35 years. Related to ↑ incidence of Ph in older patients with ALL → associated with poor prognosis.
Rarely cured with chemotherapy, although long-term survival is now routinely reported when patients are treated with combinations of chemotherapy and BCR-ABL tyrosine kinase inhibitors
Age
Overview
Common in children but can occur at any age
Philadelphia Chromosome (Ph) = 20% of adults and small percentage of children with ALL
From malignant transformation of B or T cell progenitor cells (stem cells)
Spread to lymph nodes, spleen, liver, CNS, testicles
Presence of too many lymphoblasts or lymphocytes in the BM and peripheral blood
Without treatment, ALL usually progresses quickly
Accumulation lymphoblasts BM or of extramedullary sites, in frequently immunocompromised by suppression of normal haematopoiesis
Signs and Symptoms
Abseny Cellular Maturation
Thrombocytopaenia
Anaemia
Neutropaenia
Neoplastic Infiltration
Meningeal Infiltration
Lymph Nodes
Thymus
Liver and Spleen
BM
Chronic Lymphocytic Leukaemia (CLL)
Stages
Stage 2
Too many lymphocytes in the blood, liver or spleen is larger than normal, and lymph nodes may be larger than normal
Stage 3
Too many lymphocytes in the blood and too few RBCs
Lymph nodes, liver, or spleen may be larger than normal
Stage 1
Too many lymphocytes in the blood and lymph nodes are larger than normal
Stage 4
Too many lymphocytes in the blood and too few platelets
Lymph nodes, liver, or spleen may be larger than normal or there may be too few RBCs
Stage 0
Too many lymphocytes in the blood but no other signs or symptoms of leukaemia
Slow-growing
Complications
Richter syndrome
Abnormal Ig secretion
Risk for other cancers
Risk Factors
Adult age > 55 yrs old
Family history
Description
Symptomatic or progressive CLL
Significant changes to blood counts or other serious symptoms
Recurrent CLL
Recurred after a period of time in which the cancer could not be detected
Asymptopmatic CLL
No or few symptoms
Refractory CLL
Does not get better with treatment
Aetiology
Chromosome abnormalities
Signs and Symptoms
B Symptoms
Sweating at night, fever, unintentional weight loss
Neoplastic Infiltration
Meningeal infiltration
Lymph nodes
Thymus
Liver and spleen
BM
Absent Cellular Maturation
Thrombocytopaenia
Neutropaenia
Anaemia
Pathology
Neoplastic monoclonal proliferation of mature, abnormal B lymphocytes in BM, blood, and body tissues
Onset = slow/late
Mature B lymphocytes accumulate in BM → physical suppression → prevent maturation
Others
Prognosis
Stage of cancer
CLL progresses to lymphoma or becomes prolymphocytic leukaemia
WBC count ↑ quickly
Patient's age and general health
RBC and platelet counts are ↓
CLL gets better with treatment or has recurred
Lymphocytes have spread throughout BM
Overview
Abnormal lymphocytes = leukaemia cells → not able to fight infection very well
Number of leukaemia cells increases in blood and BM → less room for healthy WBCs, RBCs, and platelets → infection, anaemia, and easy bleeding
↑ blood stem cells become abnormal lymphocytes
During or after middle age; rare in children
Cancer of blood and BM that usually gets worse slowly