Leukemia - AML, ALL, CML, CLL

Question 1

Leukemia in general
- pathophysiology
- types
- presentation
- diagnosis
- management

Answer 1

Malignant proliferation of hematopoetic blood cells => accumulate in BM that prevent maturation of normal cells

ALL - most common in children
AML
CLL - most common in adults
CML

BM failure - anemia, thrombocytopenia, neutropenia
-fatigue, SOB
-bruising, easy bleeding
-frequent infections
Neoplastic infiltration in lymphoid organs
-BM - bone pain
-thymus - mass, airway compression
-hepatosplenolymphadenopathy

Blood count, smear
BM smear
Immunophenotyping

Chemo, BM transplant, RT

Question 2

AML
- pathophysiology
- risk factors
- presentation
- diagnosis
- management

Answer 2

Immature myeloblasts (neutrophil, eosinophil, basophil, monocytes, megakaryocyte precursors) accumulate in BM => physical suppression of normal blasts

Polycythemia vera
Thrombocytosis
Myelofibrosis

Adults, children with Downs
RT, CT
MPD

Abrupt onset
- BM failure + neoplastic infiltration
- gum swelling

FBC - low RBC, PLT, low/normal/high WCC
Smear - high myeloblasts, Auer rods

Chemo, BM transplant

Question 3

ALL
- pathophysiology
- risk factors
- presentation
- diagnosis
- management
-poor prognostic factors

Answer 3

Immature lymphoblasts (B/T) accumulate in BM => physical suppression of normal blasts and release of immature lymphoblasts into blood

MOST COMMON IN CHILDREN
-Downs
-genetics
-past chemo (etoposide)

Abrupt onset
-BM failure + organomegaly (hematopoesis happens here due to BM failure), LN
-bone pain
-B symptoms

FBC - low RBC, PLT, low/normal/high WCC
BM aspirate - high lymphoblasts (20%+)
Immunophenotyping - FIND TdT
Cytogenic testing

Chemo, radiotherapy

Age U2, 10+
Male
WCC 20+ at diagnosis

Question 4

CLL
- pathophysiology
- risk factors
- presentation
- diagnosis
- management
-complications

Answer 4

Mature lymphocytes accumulate in BM => physical suppression of other cells
-majority B cells

MOST COMMON IN ADULTS

LN more marked than CML
BM failure

Blood count, smear - high lymphocytes, smudge cells
LN biopsy - lymphocytic infiltration
Immunophenotyping

Chemo, immuno, BM transplant, RT

CLL => transforms into Non-Hodgkin lymphoma

Question 5

CML
- pathophysiology
- risk factors
- presentation
- diagnosis
- management

Answer 5

Mature granulocytes accumulate in BM => physical suppression of other cells

Adults
Radiation/benzene exposuree
PHILADELPHIA CHROMOSOME (9,22)

Chronic asymptomatic phase - BM failure
Accelerated symptomatic phase - neoplastic infiltration, worsening BM failure
Blast crisis - increased blasts in blood/BM

Blood count, smear - high granulocytes
BM biopsy - hypercellularity of myeloid line
Immunophenotyping - Philadelphia

1st line - Imantinib
Hydroxyurea
Interferon a
Allogenic BM transplant

Question 6

Richter’s transformation
-what is it
-symptoms

Answer 6

Leukemia cells enter LN => high grade, fast growing non-Hodgkin lymphoma

Very unwell very quickly
-LN swelling
-fever without infection
-weight loss
-night sweats
-nausea
-abdo pain

Question 7

Management of suspected hematological malignancy in young people
-timings
-symptoms

Answer 7

Ages - 0-24
Very urgent FBC within 48hrs for leukemia

Pallor
Persistent fatigue
Unexplained fever
Unexplained persistent infections
Generalised lymphadenopathy
Persistent or unexplained bone pain
Unexplained bruising
Unexplained bleeding