Blood Cancers/Disorders Flashcards by Ben Moore

Breakdown of chronic and acute leukaemias?

Acute = rapid increase in immature blood cells which crowd out the marrow. Abnormal differentiation and excessive proliferation

Chronic = excessive build-up of abnormal but relatively mature. Normal(ish) differentiation proliferation. Mainly in older people

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Features of acute myeloid leukaemia?

Rapid proliferation of myeloblasts -> neutropenia, anaemia and thrombocytopaenia

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Rfs and presentation of AML?

RFs: incidence increases with age, Downs, irradiation and anti-cancer drugs

Bone marrow failure: infection, bleeding, pallor

Tissue infiltration: mild splenomegaly and swollen gums

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Ix for AML?

Sudan black to show Auer rods

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What is acute promyelocytic leukaemia?

Hyper aggressive subtype of AML

Genetic translocation 15:17 fuses PML with RAR-alpha

Faggot cells on cytology with lots of AUer rods

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RFs and presentation of ALL?

Most common childhood. Genetics (Downs/NF), radiation, influenza.

Thrombocytopaenia, neutropaenia and anaemia so bone marrow failure = pallor, bleeding, infections

Tissue infiltration = lymphadenopathy, hepatosplenomegaly, swollen testes and tender bones

B symptoms = fever, night sweats and weight loss

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Ix for ALL?

> 20% lymphoblasts on bone marrow biopsy

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CML features?

Hyperproliferation of granulocyte precursosrs = bone marrow failure, hypermetabolism and hyperviscosity.

Has chronic phase, accelerated phase and blast phase.

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Rfs and presentation of CML?

Rfs: M1.4:1F
Philadelphia Chr 9:22 BRC-ABL1 fusion gene

50% asymptomatic
90% massive splenomegaly
Hypermetabolic syndrome: weight loss, malaise, sweating

Bone marrow failure: anaeia, bleeding, infections

Hyperviscosity syndrome: thrombotic events, headaches

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CLL features?

Progressive accumulation of functionally incompetent lymphocytes =-> occasional features of bone marrow failure and hypermetabolism

Caused by a failure of apoptosis

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Rfs and presentation of CLL?

M:F 2:1
Genetic risk factors

50% asymptomatic, occasaionaly non-tender lymphadenopathy, occasional bone marrow failure

Usually diagnosed by routine bloods = lymphocytosis and smear/smudge cell on blood films

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What are the general investigations for leukaemia?

FBC, LDH, blood smear

Bone marrow aspirate for biopsy

Immunophenotyping or CXR

LDH is often elevated in cancer

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What is Lymphoma?

A group of blood cancers which develop from lymphocytes and the tumours are mainly found on lymph ndes

Often present with a lump/systemic B symptoms

Hodgkins, non hodgkins -> Burkits

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Rfs and presentation of Hodgkins?

Bimodal age 20-30 and >50
50% EBV associated

Painless enlarging neck mass, which can be painful after alcohol

Neutrophilia

B symptoms

Non-tender, firm and rubber lymphadenopathy with splenomegaly +- hepatomegaly

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Ix for Hodgkins?

Reed-sternberg cells on lymph node biopsy = bi-nucleate lymphocytes

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Rfs and presentation of NHL?

More common that hodgkins (85% b cell, 15% T cell or NK)

RFs: EBV, HIV, sjogrens. Incidence increases with age

Painles enlarging mass in neck, axilla or groin

B symptoms (less common than HL)

Neutropaenia
Skin rashese.g. mycosis fungoides, headache, hepatosplenomegaly (more common than HL)

NO REED STERNBERG CELLS

Features of Burkitts lymphoma?

Strong EBV association, chronic malaria reduces resistance to EBV infection ( Papua new guinea, Equatorial africa and brazil), HIV

Rapidly enlarging lymph node in the jaw

Ix for Burkitts?

Starry sky appearance under microscopy

How to stage lymphoma>

Ann Arbour stagings

What is tumour lysis syndrome?

Metabolic abnormalities from cancer treatment = especially leukaemias and lymphomas

Phosphate -> forms calcium phosphate crystals = kidney failure and hypocalcaemia

Hyperkalaemia = arrhythia

Uric acid = gout and uric acid renal stones

WHat is multiple myeloma?

Proliferation of plasma cells so production of monoclonal immunoglobulin (IgG or IgA)

features of MM?

Rfs: >70, afrocarribean
Ionising radiation, agricultural work, HIV

CRAB
Hypercalcaemia
Renal impairments: 20%, worse prognosis
Anaemia = due to crowding, frequent infections
Bone lesions = increased osteoclast = back/rib pain

Ix for MM?

Bloods = raised ESR/CRP, irea, Cr, Ca, normal ALP

Rouleaux on blood film

Bence jones proteins in serum/urine electrophoresis

Bone marrow aspirate shows increased plasma cells >10%

WHat is MGUS?

Monoclonal Gammopathy of unknown significance:

Pre-malignant condition with accumulation of some monoclonal plasma cells

1% acquire additional mutations -> MM

Absent CRAB features

What is myelodysplasia?

Def: group of syndromes where the immature blood cells do not mature normally ``` Primary = intrinsic bone marrow problem Secondary = previous chemo/radiotherapy ``` Causes chronic pancytopaenia Breakdown = refractory anaemia. refractory anaemia with ringed sideroblasts, refractory anaemia with excess blasts, refractory anaemia with excess blasts in transformation, chronic myelomonocytic leukaemia

Ix for myelodysplasia?

Bone marrow apsirate is hypercellular due to ineffective erythropoeisis

Breakdown of normal wound healing?

Primary haemostasis = platelet aggregation and plug formation depednign on patelets and vWF. Disorders are superficial bleeding Secondary haemostsis = fibrin formation to stabilise the platelet plug. Depends on clotting gfactors and disroders are deep bleeding and bruising

What is haemophilia?

Inherited disorder of clotting factor deficiency/ secondary and xling recessive a = 8 and b = 9

Presentation and Ix for haemophilia?

Haemarthrosis, haematoma and excessive bleeding and haematuria Prolnged APTT and factor assay to confirm diagnosis

What are the roles of vWF?

Platelet adhesion between endothelium and platelets (GP1b) Platelet aggregation factor 8 stabilisation

Breakdown of vWF syndrome?

1 = reduced level of normal vWF and autosomnal dominant 2 = adequte levels of defective vWF = autosomnal dominant 3= complete lack of vWF and high reduced factor 8 Autosomnal recessive

presentation of vWF syndrome?

Superficial bleeding, bruising, epistaxis, menorrhagia, prolonged gum bleeding after dental procesures, prologned bleeding from minor wounds Type 3 = deep bleeding into joints and soft tissue due to factor 8

Diagnosis of vWF syndrome?

Prolonged bleeding time Prolonged APTT and normal PT Reduced vWF Normal platelets

Breakdown of DIC?

Acute overt = emergency and life threatening. lots of bleeding Chronic non-overt = slower rate with time for compensatory response and hypercoagulable but less bleeding

DIC MOA?

Endothelial damage causes increased TF -> coagulation cascade activation = depleation of clotting factors and platelets. Concomitant activation of fibrinolysis causes bleeding into subcut tissues, mucous membraes and skin Fibrin deposits in microcirculation -> MAHA and ischaemic organ damage

DIC presentation?

Signs of underlying pathology e.g. sepsis ACUTE = bleeding issues, petechiae, ecchymosises, purpura, epistaxix, resp distress CHRONIC = clotting issues = signs of DVT or arterial thombosid

Diagnoss of DIC?

FBC = low platelets adn Hb Clotting = low fibrinogen, high fibrin degradation products, prolonged OT + APTT Blood film = shistocytes