Supportive Treat infection Manage bleeding Chemotherapy Stem cell transplantation

Often nothing/watch and wait Supportive Blood transfusions Antibiotics Cytotoxic chemotherapy Monoclonal antibodies Novel agents

Lymphoid Malignancy Flashcards by Shauneen Kiernan

What is acute lymphoblastic leukaemia?

Haematological malignancy in lymphoid progenitor/non-differentiated lymphoid cells, resulting in increased production of lymphoblasts

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In what age group is acute lymphoblastic leukaemia more common?

Most common childhood cancer

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What is the prognosis of ALL in children?

Good, 90% 5 year survival

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What is the prognosis of ALL in adults?

Poor, 30–35% 5 year survival

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What are risk factors for poor prognosis ALL?

>Age

>WCC

Slow/poor response treatment

Cytogenetics: T(9;22) and t(4;11)

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How does ALL present?

Thrombocytopenic bleeding

Bruising
Menorrhagia
Epistaxis

Lethargy/malaise

Anaemia

Dyspnoea

Arthralgia

Recurrent infection: Neutropenia

CNS infiltration

Testicular disease

Weight loss

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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

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What biopsy sign is present in ALL?

90% B lymphoblasts

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How is ALL managed?

Supportive

Treat infection
Manage bleeding

Chemotherapy

Stem cell transplantation

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Give complications of ALL

Haemorrhage

Thrombosis

Tumour lysis syndrome

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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

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What drug is used to prevent tumour lysis syndrome?

Allopurinol

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What is chronic lymphocytic leukaemia?

Malignancy characterised by monoclonal proliferation of well-differentiated/mature lymphocytes, resulting in functionally incompetent lymphocytes

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What is the most common leukaemia worldwide?

Chronic lymphocytic leukaemia

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What age group is CLL more common?

60+

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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

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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

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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

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How does Richter’s transformation present?

Lymph node swelling

Fever without infection

Weight loss

Night sweats

Nausea

Abdominal pain

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What biopsy sign is seen in CLL?

>30% lymphocytes

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What blood film sign is seen in CLL?

Smudge B cells/smear cells

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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

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How is CLL managed?

Often nothing/watch and wait

Supportive

Blood transfusions
Antibiotics

Cytotoxic chemotherapy

Monoclonal antibodies

Novel agents

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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)