Leukemia and lymphoma

Question 1

Major concerns with leukemic patient falling ill suddenly

Answer 1

1. Infection
2. Bleeding
3. Hyperviscocity
4. Tumor lysis
5. Disseminated intravascular coagulation: malignancy, sepsis, trauma, obstetric

Non-specific confusion

a. Take blood culture
b. exclude hypoglycemia
c. UEC, LFT, Ca, clotting screen
d. Consider CNS bleeding->CT/MRI

Question 2

Neutropenic regimen

Answer 2

1. Close liason with microbiologist and hematologist
2. Full barrier nursing. Hand washing.
3. Avoid IM injections->infected hematoma
4. Look for infection->mouth, axilla, perineum, IV site. Take swabs
5. Check: FBC, PLTs, INR, UEC, LFTs
6. Take cultures, urine, sputum, stool
7. CXR
8. Wash perineum after defecation
9. Oral hygeine
10. TPR obs 4 hourly
11. High calorie diet
12. Vases with roses pose a pseudomonas risk
13. Antibiotics: if febrile >38/toxic= septicemia, piperacillin+tazobactam + ceftriaxone

Question 3

What is tumor lysis syndrome, electrolyte changes, risk+, prevention

Answer 3

1. Massive cell destruction= hyperkalemia, hyperuricemia, renal impairment
2. Risk + if +LDH, +Cr, +urate, +WCC
3. Prevention with high fluids, allopurinol pre cytotoxics

Question 4

Management of DIC

Answer 4

1. Identify cause

2. Replace PLTS, cryoprecipitate (replacing fibrinogen), FFP to replace coagulation

Question 5

What is ALL, associations and epidemioloy, CNS

Answer 5

1. Malignancy of lymphoid, either T/B cell
2. Arrested maturation, proliferation of Blast cells
3. Most commonly malignancy of childhood, rare in adults
4. CNS involvement is common

Question 6

Classification systems in ALL

Answer 6

1. Morphological: L1, 2, 3
2. Immunological: Precursor B, T ALL, B ALL
3. Cytogenetic: Philaedelphia has poor prognosis

Question 7

Signs and symptoms of ALL

Answer 7

1. Marrow failure=
Anemia
Bleeding 
Infection
2. Infiltration
Hepato/splenomegaly
Lymphadenopathy
Orchidoplexy
CNS->CN palsies, menningism

Question 8

Common infections in ALL

Answer 8

1. Mouth
2. Skin
3. Perianal
4. Lung
5. Pneumocystis

Question 9

Investigations in ALL

Answer 9

1. FBC, peripheral blood smear w/ blasts, +WCC
2. CT/CXR for mediastinal
3. LP for CNS involvement

Question 10

Management of ALL

Answer 10

1. Educate and motivate
2. Supportive
   Transfusions, IV fluids, allopurinol
   Hickman line for IV access
3. Infections: neutropenia regime. IV antibiotics->co-trimoxazole to prevent Pneumocystsis, antivirals, antifungals
4. Chemotherapy
   National trials
   a. Remission induction
   b. Consolidation
   c. CNS prophylaxis
   d. Maintenance for 2 years
5. Bone marrow transplant

Question 11

Where is relapse common in ALL

Answer 11

1. Blood

2. Testes

Question 12

Prognosis: cure rates, poor prognostics

Answer 12

1. 70-90% cure in children, 40% in adults
2. Prognostics
   Male
   Adult
   Philadelphia: BCR:ABL translocation of 9:22
   CNS
   Anemic
   +WCC
   Minimal residual disease

Question 13

What is AML, incidence, asssociations

Answer 13

1. Acute myeloid leukemia
2. Most common leukemia in adults
3. Associated with radiation, MDS, Downs syndrome

Question 14

Morphological classification of AML

Answer 14

1. AML with recurrent genetic abnormalities
2. AML with multi-lineage dysplasia
3. AML therapy related
4. AML other
5. Acute leukemia of ambiguous lineage

Question 15

Is CNS involvement in AML, what about gums

Answer 15

1. CNS involvement is rare

2. Finding of gum hypertrophy

Question 16

What findings on smear is diagnostic of AML

Answer 16

Auer rods

Question 17

How is AML diagnosed

Answer 17

1. Immunophenotyping, molecular methods

2. Cytogenetic analysis

Question 18

Pitfalls in AML complication recognition

Answer 18

1. AML itself causes fever
2. Common organisms present oddly
3. Few antibodies are made
4. Rare organisms (fungi)

Question 19

Treatment for AML

Answer 19

1. Supportive as for ALL
2. Chemotherapy
3. Bone marrow transplant

Question 20

Prognosis in AML

Answer 20

1. 60% long term survival

Question 21

What is CML, epidemiology, Philadelphia chromosome, symptoms

Answer 21

1. Proliferation of myeloid cells, 15% leukemia
2. Most common in 40-60, rare in childhood
3. Ph xsome most common abnormality
4. Fatigue, weight loss, gout, fever, sweats, bleeding, abdominal discomfort

Question 22

Signs in CML and investigations

Answer 22

1. +Liver/spleen, anemia, bruising
2. ++WBC, +BEN, anemia, PLT
3. +Urate, +B12.
4. Hypercellular BM

Question 23

Natural history in CML

Answer 23

1. Survival 5-6 years

2. Chronic (months)->accelerated (+sympT, +spleen)->blast transformation

Question 24

Treatment in CML

Answer 24

1. Chemotherapy->Imatinib specific BCR:ABL tyrosine kinase inhibitor
2. Hydroxycarbamide
3. Dasatinib
4. Stem cell transplant

Question 25

What is hallmark of CLL, epidemiology, rai staging

Answer 25

1. Most common leukemia, arrested B cells->+proliferation 2. Rai stage 0= Lymphocytosis >13yr survival 1= +lymphadenopathy 8yr survival 2= +spleno/hepatomegaly 5yr 3= +anemia 2 yr survival 4= +thrombocytopenia 1 yr survival

Question 26

Typical presentation in CLL

Answer 26

1. Asymptomatic 2. Surprising finding on routine blood tests 3. Enlarged, non-tender rubbery nodes, +spleen/liver

Question 27

Investigations and complications in CLL

Answer 27

1. +Lymphocytes 2. Autoimmune hemolysis 3. Marrow infiltration->anemia, neutropenia, thrombocytopenia Complications 1. Autoimmune hemolysis 2. Marrow failure 3. +Infection due to hypogammaglobulinemia

Question 28

Management in CLL

Answer 28

1. Fldarabine + cyclophosphamide 2. Steroids in autoimmune hemolysis 3. Radiotherapy to relive lymphadenopathy/splenomegaly 4. Supportive 5. Stem cell transplant

Question 29

Prognosis in CLL`

Answer 29

1. One third never progress 2. One third will progress in time 3. One third are actively progressing

Question 30

What is lymphoma

Answer 30

1. Malignant proliferation of lymphocytes within lymph nodes, but can be in peripheral blood and infiltrate organs

Question 31

Characteristic cell of hodgkins lymphoma

Answer 31

Reed-Sternberg

Question 32

Epidemiology of HL and risk associations

Answer 32

1. Young and elderly 2. More common in males 3. Risk + if affected sibling, EBV, SLE, post transfusion, Westernisation and obesity

Question 33

Symptoms of HL

Answer 33

1. Enlarged, non tender, rubbery superficial LN->cervical, axillary, inguinal 2. Weight loss, night sweats, pruritus 3. Mass effect from mediastinal mass->SVC obstruction/bronchial 4. Alcohol induced LN pain

Question 34

Signs of HL

Answer 34

1. Weight loss, cachexia | 2. Hepato-splenoM

Question 35

Investigations in HL

Answer 35

1. FBC->anemia and low PLT 2. UEC, LFTs, LDH, Urate (cell turnover)->baseline prior to therapy commencement 3. ESR ++ 4. CXR->mediastinal mass 5. PET-CT, LP 6. Contrast CT N, chest, abdomen/pelvis (for staging) 7. Excisional LN biopsy 8. Immunohistochemistry

Question 36

Staging in HL

Answer 36

1. Confined single LN region 2. 2+ nodal areas on same side of diaphragm 3. Nodes on both sides of the diaphragm 4. Spread beyond the LN->liver or bone marrow

Question 37

Management

Answer 37

``` 1. Chemotherapy ABVD Adriamycin Bleomycin Vinblastine Dacarbazine 2. Radiotherapy ```

Question 38

General risks in radiotherapy

Answer 38

1. Increased risk of secondary malignancies->solid 2. IHD 3. Hypothyroidism 4. Lung fibrosis

Question 39

Classification of HL

Answer 39

1. Nodular sclerosing 2. Mixed cellularity 3. Lymphocyte rich 4. Lymphocyte depleted

Question 40

Causes of NHL

Answer 40

1. Congenital immunodeficiency 2. Acquired immunodeficiency 3. HIV infection 4. Infection->HTCLV-1, EBV, H pylori 5. Environmental toxins

Question 41

Is NHL just LN

Answer 41

No, can occur at other lymphoid tissue->MALT

Question 42

Signs and symptoms of NHL

Answer 42

1. Nodal disease 2. Superficial lymphadenopath 3. Extranodal: oropharynx, skin, bone, gut, CNS, lung 4. Fever, night sweats, weight loss 5. Pancytopenia: anemia, infection, bleeding

Question 43

Investigations in NHL

Answer 43

1. FBC->anemia and low PLT 2. UEC, LFTs, LDH, Urate (cell turnover)->baseline prior to therapy commencement 3. ESR ++ 4. CXR, LP 5. PET-CT 6. Contrast CT N, chest, abdomen/pelvis (for staging) 7. Excisional LN biopsy 8. Immunohistochemistry

Question 44

Chemotherapy regime in high grade NHL

Answer 44

``` RCHOP Rituximab Cyclophosphamide Hydroxydaunorubicin Vincristin (Oncovin) Prednisolone ```