Leukemia and lymphoma Flashcards
Major concerns with leukemic patient falling ill suddenly
- Infection
- Bleeding
- Hyperviscocity
- Tumor lysis
- 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
Neutropenic regimen
- Close liason with microbiologist and hematologist
- Full barrier nursing. Hand washing.
- Avoid IM injections->infected hematoma
- Look for infection->mouth, axilla, perineum, IV site. Take swabs
- Check: FBC, PLTs, INR, UEC, LFTs
- Take cultures, urine, sputum, stool
- CXR
- Wash perineum after defecation
- Oral hygeine
- TPR obs 4 hourly
- High calorie diet
- Vases with roses pose a pseudomonas risk
- Antibiotics: if febrile >38/toxic= septicemia, piperacillin+tazobactam + ceftriaxone
What is tumor lysis syndrome, electrolyte changes, risk+, prevention
- Massive cell destruction= hyperkalemia, hyperuricemia, renal impairment
- Risk + if +LDH, +Cr, +urate, +WCC
- Prevention with high fluids, allopurinol pre cytotoxics
Management of DIC
- Identify cause
2. Replace PLTS, cryoprecipitate (replacing fibrinogen), FFP to replace coagulation
What is ALL, associations and epidemioloy, CNS
- Malignancy of lymphoid, either T/B cell
- Arrested maturation, proliferation of Blast cells
- Most commonly malignancy of childhood, rare in adults
- CNS involvement is common
Classification systems in ALL
- Morphological: L1, 2, 3
- Immunological: Precursor B, T ALL, B ALL
- Cytogenetic: Philaedelphia has poor prognosis
Signs and symptoms of ALL
1. Marrow failure= Anemia Bleeding Infection 2. Infiltration Hepato/splenomegaly Lymphadenopathy Orchidoplexy CNS->CN palsies, menningism
Common infections in ALL
- Mouth
- Skin
- Perianal
- Lung
- Pneumocystis
Investigations in ALL
- FBC, peripheral blood smear w/ blasts, +WCC
- CT/CXR for mediastinal
- LP for CNS involvement
Management of ALL
- Educate and motivate
- Supportive
Transfusions, IV fluids, allopurinol
Hickman line for IV access - Infections: neutropenia regime. IV antibiotics->co-trimoxazole to prevent Pneumocystsis, antivirals, antifungals
- Chemotherapy
National trials
a. Remission induction
b. Consolidation
c. CNS prophylaxis
d. Maintenance for 2 years - Bone marrow transplant
Where is relapse common in ALL
- Blood
2. Testes
Prognosis: cure rates, poor prognostics
- 70-90% cure in children, 40% in adults
- Prognostics
Male
Adult
Philadelphia: BCR:ABL translocation of 9:22
CNS
Anemic
+WCC
Minimal residual disease
What is AML, incidence, asssociations
- Acute myeloid leukemia
- Most common leukemia in adults
- Associated with radiation, MDS, Downs syndrome
Morphological classification of AML
- AML with recurrent genetic abnormalities
- AML with multi-lineage dysplasia
- AML therapy related
- AML other
- Acute leukemia of ambiguous lineage
Is CNS involvement in AML, what about gums
- CNS involvement is rare
2. Finding of gum hypertrophy
What findings on smear is diagnostic of AML
Auer rods
How is AML diagnosed
- Immunophenotyping, molecular methods
2. Cytogenetic analysis
Pitfalls in AML complication recognition
- AML itself causes fever
- Common organisms present oddly
- Few antibodies are made
- Rare organisms (fungi)
Treatment for AML
- Supportive as for ALL
- Chemotherapy
- Bone marrow transplant
Prognosis in AML
- 60% long term survival
What is CML, epidemiology, Philadelphia chromosome, symptoms
- Proliferation of myeloid cells, 15% leukemia
- Most common in 40-60, rare in childhood
- Ph xsome most common abnormality
- Fatigue, weight loss, gout, fever, sweats, bleeding, abdominal discomfort
Signs in CML and investigations
- +Liver/spleen, anemia, bruising
- ++WBC, +BEN, anemia, PLT
- +Urate, +B12.
- Hypercellular BM
Natural history in CML
- Survival 5-6 years
2. Chronic (months)->accelerated (+sympT, +spleen)->blast transformation
Treatment in CML
- Chemotherapy->Imatinib specific BCR:ABL tyrosine kinase inhibitor
- Hydroxycarbamide
- Dasatinib
- Stem cell transplant
What is hallmark of CLL, epidemiology, rai staging
- Most common leukemia, arrested B cells->+proliferation
- Rai stage
0= Lymphocytosis >13yr survival
1= +lymphadenopathy 8yr survival
2= +spleno/hepatomegaly 5yr
3= +anemia 2 yr survival
4= +thrombocytopenia 1 yr survival
Typical presentation in CLL
- Asymptomatic
- Surprising finding on routine blood tests
- Enlarged, non-tender rubbery nodes, +spleen/liver
Investigations and complications in CLL
- +Lymphocytes
- Autoimmune hemolysis
- Marrow infiltration->anemia, neutropenia, thrombocytopenia
Complications
- Autoimmune hemolysis
- Marrow failure
- +Infection due to hypogammaglobulinemia
Management in CLL
- Fldarabine + cyclophosphamide
- Steroids in autoimmune hemolysis
- Radiotherapy to relive lymphadenopathy/splenomegaly
- Supportive
- Stem cell transplant
Prognosis in CLL`
- One third never progress
- One third will progress in time
- One third are actively progressing
What is lymphoma
- Malignant proliferation of lymphocytes within lymph nodes, but can be in peripheral blood and infiltrate organs
Characteristic cell of hodgkins lymphoma
Reed-Sternberg
Epidemiology of HL and risk associations
- Young and elderly
- More common in males
- Risk + if affected sibling, EBV, SLE, post transfusion, Westernisation and obesity
Symptoms of HL
- Enlarged, non tender, rubbery superficial LN->cervical, axillary, inguinal
- Weight loss, night sweats, pruritus
- Mass effect from mediastinal mass->SVC obstruction/bronchial
- Alcohol induced LN pain
Signs of HL
- Weight loss, cachexia
2. Hepato-splenoM
Investigations in HL
- FBC->anemia and low PLT
- UEC, LFTs, LDH, Urate (cell turnover)->baseline prior to therapy commencement
- ESR ++
- CXR->mediastinal mass
- PET-CT, LP
- Contrast CT N, chest, abdomen/pelvis (for staging)
- Excisional LN biopsy
- Immunohistochemistry
Staging in HL
- Confined single LN region
- 2+ nodal areas on same side of diaphragm
- Nodes on both sides of the diaphragm
- Spread beyond the LN->liver or bone marrow
Management
1. Chemotherapy ABVD Adriamycin Bleomycin Vinblastine Dacarbazine 2. Radiotherapy
General risks in radiotherapy
- Increased risk of secondary malignancies->solid
- IHD
- Hypothyroidism
- Lung fibrosis
Classification of HL
- Nodular sclerosing
- Mixed cellularity
- Lymphocyte rich
- Lymphocyte depleted
Causes of NHL
- Congenital immunodeficiency
- Acquired immunodeficiency
- HIV infection
- Infection->HTCLV-1, EBV, H pylori
- Environmental toxins
Is NHL just LN
No, can occur at other lymphoid tissue->MALT
Signs and symptoms of NHL
- Nodal disease
- Superficial lymphadenopath
- Extranodal: oropharynx, skin, bone, gut, CNS, lung
- Fever, night sweats, weight loss
- Pancytopenia: anemia, infection, bleeding
Investigations in NHL
- FBC->anemia and low PLT
- UEC, LFTs, LDH, Urate (cell turnover)->baseline prior to therapy commencement
- ESR ++
- CXR, LP
- PET-CT
- Contrast CT N, chest, abdomen/pelvis (for staging)
- Excisional LN biopsy
- Immunohistochemistry
Chemotherapy regime in high grade NHL
RCHOP Rituximab Cyclophosphamide Hydroxydaunorubicin Vincristin (Oncovin) Prednisolone
