Leukaemia

Question 1

Acute problems in leukaemia

Answer 1

Infection (cultures, hygiene, Abx)
Bleeding (platelets ±FFP)
Hyperviscosity/DIC
Tumour lysis syndrome (inc K, inc Urate, AKI)

Question 2

MASCC score

Answer 2

Multinational Association for Supportive Care in Cancer score used to assess risk of serious complications in febrile neutropenia
Score ≥21 low risk, can avoid admission

Question 3

Acute leukaemia neutropenia regimen

Answer 3

Avoid IM injection
Clean mouth and anus following defecation

High calorie diet

Look for and swab infection
Bloods and cultures

If ≥38˚C or ≥37.5 2 times within 1 hr or pt septic, pip-taz (±vancomycin) until afebrile for 72hrs or 5 day course + no longer neutropenic

Question 4

What is ALL

Answer 4

Malignancy of T/B cell lineages, uncontrolled proliferation and no differentiation of immature blast cells
Marrow failure + tissue infiltration
CNS involvement common

Question 5

ALL classification

Answer 5

Morphological (FAB system) by microscopic appearance L1/L2/L3
Immunological, surface markers say B/T/B precursor
Cytogenetic, chromosome analysis good for predicating prognosis + recurrence

Question 6

ALL presentation

Answer 6

Most common childhood cancer
Marrow failure (dec Hb, dec WCC, dec platelets)
Infiltration (hepato/splenomegaly; lymphadenopathy; CNS involvement e.g. CN palsy, meningism)
Common infections e.g. bacterial septicaemia, zoster, CMV, measles, candidiasis, Pneumocystis pneumonia

Question 7

ALL tests

Answer 7

Characteristic blast cells on blood film + bone marrow
WCC usually high
CXR/CT for mediastinal + abdo lymphadenopathy
LP for CNS involvement

Question 8

ALL management (excluding chemo)

Answer 8

Supportive: Blood transfusion, IV fluids, allopurinol (prevents tumour lysis syndrome)
Infections: Neutropenic regimen ± prophylactic anti fungals/virals/biotics
Allogenic marrow transplants once in 1st remission for young adults
Haem remission: None in blood, normal/recovering FBC, <5% blasts in normal regenerating marrow

Question 9

ALL chemo management

Answer 9

Remission induction: e.g. vincristine, pred, l-asparaginase, daunocrubicin
Consolidation: High-dose therapy blocks over weeks
Maintenance: prolonged chemo for 2yrs (e.g. mercaptopurine daily, methotrexate weekly, vincristine + prednisolone monthly); examine testes/CNS/blood for relapse

CNS prophylaxis: intrathecal methotrexate ± CNS irradiation

Question 10

ALL prognosis

Answer 10

70-90% cure for children, 40% for adults
Poorer prognosis if adult, male, Philadelphia chromosome, CNS signs, dec HB, WCC >100, B-cell ALL, relapse (unless marrow transplant)

Question 11

What is AML

Answer 11

Neoplastic proliferation of blast cells from marrow myeloid cells, rapidly progressing (2mth death if untreated)

Question 12

AML morphological classification

Answer 12

AML with recurrent genetic abnormalities
AML multi-lineage dysplasia (e.g. 2˚ to myelodysplastic syndrome)
AML due to therapy (e.g. from previous cytotoxics)
AML other

Question 13

AML presentation

Answer 13

Marrow failure: Anaemia, infection, bleeding, DIC (in promyelocytic subtype)
Infiltration: hepato/splenomegaly, gum hypertrophy, skin

Question 14

AML diagnosis

Answer 14

WCC often raised but not always
Immunophenotyping + molecular analysis (cytogenetics guides treatment)
Bone marrow shows Auer rods in biopsy

Question 15

AML complications

Answer 15

Infection + septicaemia (often fungi)
AML itself causes fever
Leukostasis if v high WCC

Question 16

AML treatment

Answer 16

Supportive as in ALL
Chemo (daunorubicin and cytarabine (inc susceptibility if RAS mutation as seen in 20%) mainly), 5 cycles of 1wk blocks
BMT

Question 17

Bone marrow transplant steps

Answer 17

Cyclophosphamide and irradiation to destroy host marrow (immune system)
Allogeneic HLA-matched marrow with cyclosporin ± methotrexate to reduce GVHD
Autologous BMT occasionally used in intermediate prognosis

Question 18

What is myelodysplastic syndrome

Answer 18

Bone marrow failure mostly primary but can be due to chemo/radiotherapy
Pancytopenia + dec reticulocytes

Question 19

Myelodysplastic syndrome treatment

Answer 19

Treat with transfusions as needed
EPO ± G-CSF may reduce transfusion burden
Allogeneic stem cell transplant if young
Thalidomide analogues/ hypomethylating drugs (azacitidine/decitabine) for end of life quality

Question 20

What is myelofibrosis

Answer 20

Myeloproliferative disorder caused by hyperplasia of abnormal megakaryocytes

Question 21

Myelofibrosis features

Answer 21

Haematopoiesis in liver + spleen
Thrombocytopenia, leucopenia in progressive disease
Tear-drop RBCs seen in blood film