Lymphoid Malignancy Flashcards
What is acute lymphoblastic leukaemia?
Haematological malignancy in lymphoid progenitor/non-differentiated lymphoid cells, resulting in increased production of lymphoblasts
In what age group is acute lymphoblastic leukaemia more common?
Most common childhood cancer
What is the prognosis of ALL in children?
Good, 90% 5 year survival
What is the prognosis of ALL in adults?
Poor, 30–35% 5 year survival
What are risk factors for poor prognosis ALL?
>Age
>WCC
Slow/poor response treatment
Cytogenetics: T(9;22) and t(4;11)
How does ALL present?
Thrombocytopenic bleeding
- Bruising
- Menorrhagia
- Epistaxis
Lethargy/malaise
Anaemia
Dyspnoea
Arthralgia
Recurrent infection: Neutropenia
CNS infiltration
Testicular disease
Weight loss
What investigations are used in ALL diagnosis?
Bone Marrow Biopsy
FBC, within 48 houts in young people with suspicious symptoms
- Increased WCC
Clotting
Blood film
What biopsy sign is present in ALL?
90% B lymphoblasts
How is ALL managed?
Supportive
- Treat infection
- Manage bleeding
Chemotherapy
Stem cell transplantation
Give complications of ALL
Haemorrhage
Thrombosis
Tumour lysis syndrome
What is tumour lysis syndrome?
Cell break down secondary to chemotherapy, resulting in the release of intracellular compounds such as potassium, phosphate and uric acid, resulting in renal failure
What drug is used to prevent tumour lysis syndrome?
Allopurinol
What is chronic lymphocytic leukaemia?
Malignancy characterised by monoclonal proliferation of well-differentiated/mature lymphocytes, resulting in functionally incompetent lymphocytes
What is the most common leukaemia worldwide?
Chronic lymphocytic leukaemia
What age group is CLL more common?
60+
What are the poor prognostic factors for CLL?
Advanced disease (Binet B or C)
Atypical lymphocyte morphology
Rapid lymphocyte doubling time (<12 months)
CD38+ expression
Loss/mutation p53; del 11q23 (ATM gene)
Unmutated IgVH gene status
How does CLL present?
Often asymptomatic at presentation
Recurrent infection
Weight loss
Bleeding
Sweating
Malaise
Abdominal discomfort
Lymphadenopathy, more marked than CML
Splenomegaly
Hepatomegaly
Petechiae
Pallor
What investigations are used in CLL?
Immunophenotyping
Bone marrow biopsy
Lymph node biopsy
FBC
- Warm autoimmune haemolytic anaemia
- Lymphocytosis
Blood film
- Smudge B cells/smear cells
How does Richter’s transformation present?
Lymph node swelling
Fever without infection
Weight loss
Night sweats
Nausea
Abdominal pain
What biopsy sign is seen in CLL?
>30% lymphocytes
What blood film sign is seen in CLL?
Smudge B cells/smear cells
What are the indications for treatment of CLL?
Progressive bone marrow failure
Massive lymphadenopathy
Progressive splenomegaly
Lymphocyte doubling time <6 months or >50% increase over 2 months
Systemic symptoms
Autoimmune cytopenias
How is CLL managed?
Often nothing/watch and wait
Supportive
- Blood transfusions
- Antibiotics
Cytotoxic chemotherapy
Monoclonal antibodies
Novel agents
Give complications of CLL
Transformation to high-grade lymphoma/Richter’s transformation
Autoimmune warm haemolytic anaemia
Idiopathic thrombocytopenic purpura
Hypogammaglobulonaemia
Give examples of Monoclonal antibodies used in CLL treatment
Rituximab
Obinatuzumab
What staging classification is used for CLL?
Binet
Describe Binet stage A
<3 lymph node areas
Describe Binet stage B
3 or more lymph node areas
Describe Binet stage C
Stage B plus anaemia or thrombocytopenia
What is the prognosis of Binet stage A?
Same as age matched controls
What is the prognosis of Binets stage B?
8 years
What is the prognosis of Binet stage C?
6 years
What is Richter’s transformation?
Occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma, causing patients to become unwell very suddenly
What is Hodgkin’s lymphoma?
Hodgkin’s lymphoma is a malignant proliferation of lymphocytes characterised by the presence of the Reed-Sternberg cell
Give types of hodgkins lymphoma
Nodular scleroising, most common
Mixed cellularity
Lymphocyte predominant, best prognosis
Lymphocyte depleted, worst prognosis
What age does Hodgkin’s peak at?
Bimodal age distribution
- 1st peak at 15-35 years
- 2nd peak 50-60
How does Hodgkin’s present?
Painless, non-tender, asymmetrical lymphadenopathy
Weight loss
Pruritus
Night sweats
Fever
alcohol pain
Normocytic anaemia, eosinophilia
LDH raised
What is the most common site of lymphadenopathy in Hodgkin’s?
Cervical chain
What cells are characteristic of Hodgkin’s?
Reed-Sternberg cells (mirror image nuclei)
How is Hodgkin’s managed?
Combination chemotherapy
Radiotherapy
Monoclonal antibodies: Anti-CD30
Immunotherapy
What staging system is used in lymphoma?
Ann Arbor system
With A or B to indicate the presence or absense of B symptoms
Describe stage 1 lymphoma
One node affected
Describe stage 2 lymphoma
More than one node affected on the same side of the diaphragm
Describe stage 3 lymphoma
Nodes affected on both sides of the diaphragm
Describe stage 4 lymphoma
Extra-nodal involvement such as spleen, bone marrow or CNS
Give an example of a low grade non-hodgkin’s lymphoma subtype
Follicular, marginal zone
Give examples of high grade non-hodgkin’s lymphoma sub-types
Diffuse large B cell lymphoma/Burkitt’s lymphoma
T cell lymphoma
What is the prognosis of non-hodgkins lymphoma?
Low-grade has a better prognosis
High-grade has a worse prognosis but a higher cure rate
Give risk factors for non-hodgkin’s
Elderly
Caucasians
FH
Viral infection
- EBV, Burkitt’s
- HSV
Immunosuppression
- HIV, Burkitt’s
- DM
- Transplant
History of chemotherapy or radiotherapy
Autoimmune disease
- SLE
- Coeliac
How does non-hodgkin’s present?
Lymphadenopathy
Hepatosplenomegaly/palpable abdominal mass
B Symptoms occur later than hodgkins
Testicular mass
Weight loss
Fever
Extra nodal disease is more common
What investigations are used in non-hodgkins lymphoma diagnosis?
Excisional node biopsy
- Starry sky appearance in Burkitt’s
CT chest, abdomen and pelvis for staging
HIV test
FBC and blood film
ESR useful as a prognostic indicator
LDH useful as a prognostic indicator
What is the management of non-hodgkins lymphoma?
Watchful waiting
Chemotherapy
Radiotherapy
All patients will receive flu/pneumococcal vaccines
Patients with neutropenia may require antibiotic prophylaxis
Consider autologous bone marrow transplantation in unresponsive or recurrent disease
Give complications of non hodgkins lymphoma
Bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia
Superior vena cava obstruction
Metastasis
Spinal cord compression
Complications related to treatment
What is the most common lymphoma?
High grade Non-Hodgkin lymphoma is most common lymphoma, with diffuse large B-cell lymphoma most common subtype of lymphoma
Give complications of tumour lysis syndrome
hyperkalaemia
hyperphosphataemia
hypocalcaemia
hyperuricaemia
acute renal failure
What is given before chemotherapy to prevent tumour lysis syndrome?
Rasburicase
- Recombinant version of urate oxidase, an enzyme which catalyses the conversion of uric acid to allantoin, increasing renal extraction
How does neutropenic sepsis present?
Commonly occurs 7-14 days after chemotherapy
Neutrophil count of < 0.5 * 109
Temperature higher than 38ºC
Anorexia
Malaise
Lethargy
Sweats
Fever
Chills
How is neutropenic sepsis managed?
Broad spectrum antibiotics immediately
- Tazocin (Piperacilin and Tamzobactin) + Gentamicin
- Alternate therapy if still febrile after 48 hours
G-CSF (Granulocyte Colony Stimulating Factor)
