Hematological Malignancy Flashcards

1
Q

How can the stage of haemopoiesis affect the cancer’s aggression

Name of cancers based on each cell in haemopoiesis

  • blasts
  • mature RBC, platelets, granulocytes
  • plasma
  • mature lymphocytes
A

Precursor cells - acute (more aggressive)
Mature cells - chronic (less aggressive

Blasts - acute leukemia
Mature RBC, platelets, granulocytes - MPNs
Plasma - myeloma
Maturę lymphocytes - B, T, NK lymphomas

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2
Q

What is the main defining feature of acute leukemias

2 main types of acute leukemia and their main characteristics

  • acute lymphoblastic leukemia
  • acute myeloid leukemia
A

20%+ blasts in blood or BM

Acute lymphoblastic leukemia

  • childhood cancer but incidence increases with age
  • curable in children but less in adults

Acute myeloid leukemia

  • elderly cancer
  • prognosis depends on genetic abnormalities
  • an umbrella term that covers leukemic changes at any point in hematopoiesis
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3
Q

Presentation of acute leukemias

  • pathophysiology
  • past medical history
A

Dysfunctional leukoblasts are made in the BM, not released => affects other cell lines and leukemia cells leak out

Abrupt onset
Loss of BM function - pancytopenia
-anemia, fatigue
-fever, infection
-bruising, easy bleeding

Organ infiltration - hepatomegaly, splenomegaly
-lymphadenopathy in ALL

CNS - headache, nausea, nerve palsy

AML can have strong FHx
MPN, MDS can develop into AML

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4
Q

Investigations to diagnose acute leukemia (ALL, AML)

A

Blood and BM morphology
Immunophenotyping - identify antigens present
Karyotyping - chromosomal abnormalities
Genetic analysis - assess for mutations

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5
Q

Management of acute leukemias

A

Combination chemotherapy with repeated courses

Allogenic hemopoietic stem cell transplant if

  • relapse
  • poor prognosis

Treatment guided by flow cytometry, PCR, sequencing

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6
Q

Pathophysiology of tumour lysis syndrome

A

Rapid release of cellular components into circulation after rapid destruction of rapidly proliferating malignant cells

Release of DNA => increased uric acid
Release of Ca, PO4 => arrythmias
Both can lead to AKI

Hydration
Allopurinol (xanthine oxidase) => prevent uric acid formation
Monitor K, manage if needed
Dialysis is an option

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7
Q

Acute promyelocytic leukemia

  • pathophysiology
  • management
A

Aggressive form of AML

MEDICAL EMERGENCY

  • promyelocytes release cytokines => DIC
  • platelet production low => bleed risk

Can manage with vitamin A analogues and arsenic trioxide

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8
Q

What are lymphoid neoplasms

A

Clonal tumours of B, T, NK cells

-malignancy can form at any stage of development and maturation

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9
Q

Myeloma

  • epidemiology
  • pathophysiology
  • presentation
A

BM plasma cell neoplasm => excess IgG, IgA released into serum, urine

  • can arise from MGUS
  • 60-70 years

Bone pain, lesions, osteoporosis => high Ca
Fatigue, infection
Renal failure from high Ca, dehydration, amyloid

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10
Q

Myeloma

  • investigations
  • diagnosis
A

IgG, IgA monoclonal proteins in serum, urine
Increased plasma cells in BM

Whole body MRI - bone lesions

BM aspirate - identify myeloma cells

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11
Q

Presentation of myeloma in kidneys

A

Light chains block tubules => form cast nephropathy

Light chain accummulate in glomerulus => nephrotic syndrome
-can accumulate in other areas => macroglossia, peripheral neuropathy, HF, GI, liver

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12
Q

Describe the impact myeloma can have on the spinal cord

-management

A

SC compression - sensory loss, paraesthesia, limb weakness, difficulty walking, sphincter changes

Management
Supportive - bed rest, backbrace

Imaging - urgent MRI

Medical - high dose steroids to reduce plasma cell burden

If soft tissue lesion - local radio
If bony compression/SC unstable - surgery

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13
Q

What is hyperviscosity syndrome

  • presentation
  • management
A

High levels of paraprotein => increase viscosity => reduces O2 delivery => end organ dysfunction

Bleeding into skin, retina
CNS - headache, blurred vision, poor conc, low GCS, tinnitus, seizures
CV - HF, intermittent claudication
Resp - pulmonary congestion, SOB

Treat underlying disease
DO NOT TRANFUSE RED CELLS
-plasmapheresis
-isovolemic venesection with saline replacement

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14
Q

Myeloma management

A

Chronic disease - only treat supportively if symptomatic

  • bisphosphonates - bone protection
  • radio - pain, palliation
  • repeated chemo/steroids/meds
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15
Q

What are the main 2 types of lymphoma

-risk factors

A

Replacement of normal lymphoid structures with malignant cells

Can be nodal/extranodal
Hodgkin - 20s and increased incidence with age
Non Hodgkin - mainly associated with age
-B cell most common

Risk factors

  • increasing age
  • viruses (EBV, HIV, HepB, C)
  • chronic inflammation (Hpylori, AI)
  • IC (organ transplant, meds)
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16
Q

Presentation of lymphoma

A

Fever, night sweats, weight loss, fatigue
Lympadenopathy
Hepatosplenomegaly
Symptoms in extranodal sites

Hodgkin

  • itch or alcohol induced pain
  • mediastinal mass => SVC obstruction, SOB
  • cyclical changes in fever (PelEbstein)
17
Q

Diagnosis and staging of lymphoma

A

LN biopsy
Stage
-CT/PET
-BM aspirate - assess liquid, can be used to immunophenotyping, cytogenetics
-BM trephine - assess solid section of bone for immunohistochemistry

Also can assess for genetic abnormalities that will guide diagnosis and treatment

18
Q

Treatment of lymphomas in general

Treatment of Hodgkin lymphoma

A

High grade - urgent with curative intent
Early low grade - radio with curative intent

Symptomatic low grade - treat
Asymptomatic low grade - wait

Gastric MALT (caused by HPylori) can be cured => HPylori eradication

Hodgkin lymphoma treatment depends on staging

  • cycles of chemo and radio
  • monoclonal AB
  • stem cell rescue can be used for relapse
19
Q

What is autologous stem cell transplant

Why is this used in chemotherapy

A

For myeloma, Hodgkin and high grade lymphoma
Remove and freeze patient’s own stem cells
Chemo destroys the patient’s normal hemopoetic tissue => pancytopenia

Thawed stem cells are reinfused to aid recovery of BM

20
Q

What are the side effects of chemotherapy

A
N+V+D
Hair loss
Myelosuppression, neutropenic sepsis
Fatigue
Lung, nerve damage, cardiomyopathy
Infertility
Secondary cancer
Steroid related side effects
21
Q

What is neutropenic sepsis

-what is the general management

A

ONCOLOGICAL EMERGENCY
Fever and neutrophil count U0.5x10^9
Broad spec ABx with G-ve coverage
-tazocin

22
Q

What is diffuse large B cell lymphoma

What is follicular lymphoma

A

Most common lymphoma
High grade CD20+ tumour
Common in older adults, males

Most common low grade lymphoma
CD20+
Only treated when symptomatic or early
-chemo + rituiximab
Common in older adults, females
23
Q

Characteristics of chronic lymphocytic leukemia

A

Proliferation of mature B cells in BM, LN and spill out into blood
Often accidentally found

Risk factors
-increased age, male

Presentation

  • cancer red flags
  • recurrent infection
  • AI hemolytic anemia, thrombocytopenia
  • BM failure
  • lymphadenopathy
  • splenomegaly
24
Q

MPN pathophysiology

Polycythemia vera presentation

A
BM making too many blood cells
PV - too many RBCs, neutrophils, platelets
-JAK2 common
-hyperviscosity
-splenomegaly
-easy bleeds
-plethoric
25
MPN - essential thrombocytopenia - presentation - pathophysiology
Megakaryocyte proliferation => too many platelets -JAK2 common - burning feeling in hands - high platelets - thrombosis and haemorrhage
26
MPN - myelofibrosis - pathophysiology - presentation
Hyperplasia of abnormal megakaryocytes => BM fibrosis => dysfunctional blood cells made in BM => haematopoiesis occurs in liver and spleen but cannot make enough RBCs to keep up with demand Anemia, fatigue Massive splenomegaly Weight loss, night sweats
27
MDS pathophysiology
Abnormal growth of blood cells that accumulate in BM => spill out into blood - pancytopenia - splenomegaly