ALL
- what cells are affected?
- What is the patho genesis?
- Who does it occur in?
- What would you see on investigations?
- Clinical presentation? What about with T cell ALL?
- Tx?
- Survival rate?
ALL: acute lymphoid leukaemia
- early lymphoid progeny (stem cell): B-cell progeny is most common
- Common in children, then second wave >40yo
- No specific genetic risk but higher incidence in Down syndrome, or pt previously had chemo
Patho:
- Maturation arrest ~ no further differentiation of lymphoid progeny
- Cells divide rapidly & invade BM —> BM suppression
- Cells infiltrate blood & metastasize
Investigations
- BM suppression: anemia, neutropenia, thrombocytopenia, lymphocytosis
- Smear/BM biopsy ~ lymphoblasts
- Immunophenotyping ~ used to distinguish AML from ALL
Clinical presentation
- Abrupt, stormy onset
- Sx BM suppression: anemia, thrombocytopenia, neutropenia
- Bone sx: bone pain
- Lymph node sx: lymphadenopathy
T-cell ALL —> thymus enlargement & compression of mediastinal structures
Tx
- induction chemo —> intensification —> maintenance —> prophylactic
Good survival rate in children
AML
What is it/Patho?
Who does it occur in?
Investigations?
Clinical Presentation?
Acute Myeloid Leukemia
Arrest of differentiation of myeloid progeny —> excessive cell division —> suppresses BM —> infiltrates blood —> blasts travel to other sites and are deposited —> also can cause tumour lysis syndrome + leukostasis
Occurs in older adults, Down syndrome
Investigations
- BM biopsy & smear = >20% myeloblasts
— myeloblasts would have Auer rods
- CBC: anemia, thrombocytopenia, neutropenia
Clinical Presentation
- anemia sx, thrombocytopenia sx, neutropenia sx
- Bone pain
- Leukemia cutis
- Gum swelling
Tx
- induction
Consolidation
What is leukostasis?
Where have a large # of myeloblasts that interfere with circulation —> leading to ischemia - hypoxemia & bleeding
Common sx:
- pulmonary infiltrates & respiratory distress
- CNS bleeding
- altered mental status
- priapism ~ prolonged penile erection
Tx
- hydration, avoid diuretics, RBC transfusion
What is tumour lysis syndrome?
Complication of tx cancer
Lysis of malignant cells —> release their contents into bloodstream
- hyperK+ ~ arrhythmia , hyperPO4, high levels Uric acid ~ azotemia ~ renal failure, lactic acidosis, hypocalcemia bc high PO4 ~ binding of both
Hypoca2+ —> tetany ~ involuntary m. Contractions, ∆ mental status, muscle weakness
CLL
E/E
Patho
Investigations
Clinical Presentation
Treatment
Chronic Lymphocytic Leukemia
- Common in elderly, >75 yo
- Lazy, neoplastic division of mature B cells in BM & blood
Patho:
- mature B lymphocytes accumulate in BM, blood, spleen, lymph nodes
- eventually they displace normal hematopoisis
Clinical Presentation
- Asx (caught on regular blood work)
- B sx
- sx associated with anemia, thrombocytopenia, neutropenia
- sx associated with neoplastic infiltration: lymph nodes, spleen, liver, BM
- Bc B cells ~ auto-antibodies ~ autoimmune hemolytic anemia
Investigations
- neutropenia, anemia, thrombocytopenia, +++lymphocytes
- BM aspiration/biopsy: lymphocytes
- smear: lymphocytes & smudge cells
Tx
- asx: no treating
- sx: chemo/stem cell transplant
CML
E/E
Patho
Investigations
Clinical Presentation
Tx
Chronic myeloid Leukemia
Increased proliferation of granulocytic cells without loss of their ability to differentiate (neutrophil, basophils, eosinophils)
Can be any age groups & usually involves Philadelphia chromosome (9 & 22)
Philadelphia chromosome allows uncontrolled proliferation of myeloid cells & so mature granulocytes accumulate in BM & can cause displacement of normal hematopoiesis
Investigations
- thrombocytopenia, anemia
Increased granulocytes
- smear: granulocytes
Clinical presentation
- Chronic phase: asx
- Accelerated phase ~ splenomegaly, pruritus
- Blast crisis (>20% blasts): active Leukemia, constitutional sx
Tx
- depends on phase: stem cell transplant, imatinib
Can progress to AML bc high chance of future mutations since you have continue division of myelocytes
Hogkin’s Lymphoma
E/E
Patho
Investigations
Clinical presentation
Tx
Neoplastic proliferation of lymphoid cells in lymph node (germinal site ~ where they’re made) AND usually form Reed-Sternberg cells
E/E - bimodal (20 yo, >50 yo)
Assoc. EBV, immunodeficiency
Pathophys:
- unregulated cell division of lymphocytes in germinal center
- formation of Reed-Sternberg cells = secrete cytokines
- Cytokines attract other immune cells (neutrophils, lymphocytes, monocytes) ~ help form the majority of the tumour
- spreads contiguously ~ from 1 adjacent lymph node to another
Investigations
- Reed Sternberg cells (Owl eyes)
Clinical Presentation
- asymptomatic lymphadenopathy (painless)
- B symptoms
- hepatosplenomegaly
tx
- Good prognosis
- chemo
Non Hodgkins Lymphoma
E/E
Patho
Investigations
Clinical presentation
Tx
Malignant proliferation of lymphoid cells in lymphoid tissue (B or T cells)
Increases with age, chromosomal translocations, EBV, immunodeficiency
Types
- B cell: follicular (indolent), diffuse large B cell (aggressive), Burkett (highly aggressive), Mantle cell (aggressive, marginal zone (indolent), lymphoplasmacytic lymphoma (indolent)
- T cell: adult T cell (aggressive), mycoides fungoides (aggressive)
Patho:
- proliferation of lymphocytes in lymphoid tissue
- uncontiguous spread to lymph nodes and extra nodal sites
Clinical Sx
- painless lymphadenopathy incontiguous
- extra nodal spread: GI - obstruction, BM - suppression, Neuro - spinal cord compression)
- B sx, hepatosplenomegaly
Febrile neutropenia
Occurs in chemo pts but can also occur in pt with heme malignancies
T>38 >1 hr PLUS neutrophil count <0.5
Work up
CBC, blood culture x2, urinalysis & culture, ESR/CRP, INR, lactate, BMP, type & screen
Tx
- broad spectrum beta-lactation PO PLUS empiric anti fungal if fever >3-7 d
