Week 4 - Haematology Flashcards by niamh cummings

Where would you find sites of hemopoiesis in adults?

Bone marrow of: ribs, vertebrae, sternum, skull, pelvis

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What is Leukemogenesis?

A multi step process. When the neoplastic cells is a heamopoietic cell in early myeloid or lymphoid. There is dysregulation of cell growth and differentiation. Proliferation of leukaemia clone with differentiation blocked at an early stage. So cells can’t mature- bone marrow failure.

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What is Essential Thrombocytosis?

Sustained megakaryocytic proliferation, leading to an increase in the number of circulating platelets.

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What are the complications of a major haemorrhage?

Acidosis, Hypothermia, Hypocalcaemia, coagulopathy

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What testifies a myeloma?

Increased plasma cells in bone marrow.
Clonal Ig or paraprotein
Lytic bone lesions

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What is the gold standard treatment for fanconi anaemia?

Allogenic SC transplant

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What cells are characteristic in Hodgkins lymphoma?

Reed stern burg cells

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What presentation is characteristic in meningitis?

Pyrexia and headache

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What factors are in teh extrinsic pathway?

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What factors are in the intrinsic pathway?

8, 9, 11, 12

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What is inherited thrombophilia?

Deficiencies of natural anticoagulant (protein S, protein C, antithrombin)

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Give an example of a JAK2 inhibitor

Ruxolitinib

For myeloproliferative disorders

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What is anaemia of chronic disease

Failure of iron utilisation. iron trapped in RES

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What are some reasons for Hypochromic microcytic RBCs?

IDA - not enough harm
Thalassaemia - Not enough global
ACD
Sideroblastic anaemia - iron doesn’t get incorporated into haem

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Define major haemorrhage

Loss of more than 1 blood volume within 24 hours

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What are some hereditary conditions impairing bone marrow function?

Sickle cell anaemia, thalassaemia, fanconi anaemia, dyskeratosis congentia

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What are some acquired conditions impairing bone marrow function?

aplastic anaemia, leukaemia, metastatic malignancy, infections, drugs and toxins.
Chemo, haematinic diseases

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What is a myeloproliferative disorder?

Clonal disorder of hemopoesis.
Increase in the number of one or more blood progeny
JAK2 or Calreticulin. Can transform into AML

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What is fanconi anaemia?

Genetic disease due to an impaired response to DNA damage. FANCA-G. AR

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What are the types of allogenic sc transplants?

Full intensity ‘myeloblastive’ - a severe form of myelosuppression. (conditioned with high dose chemo)
Reduced intensity ‘mini-transplant’ - in older pts (conditioned with low dose chemo)

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How may GvHD present

Skin rash, jaundice or diarrhoea
Acute - within first 100 days
Chronic - after first 100 days

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What are some problems with SC transplants?

GvHD, limited donor availability, age limit = 65, hypothyroidism, infertility, cataracts, osteoporosis, relapse.

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What is sideroblastic anaemia?

Iron doesnt get incorporated into haem.

Hypochromic microcytic

What are some effects of B12 deficiency?

Bilateral peripheral neuropathy
Demyelination of the posterior and pyramidal tracts of the spinal cord.
Beefy red Tongue, pallor

Define Major haemorrhage.

Loss of more than one blood volume in 24 hours

Explain how you would perform the PT test?

Add the patients plasma to thromboplastin (TF and phospholipids), warm to 37 and add Ca. Time for long it takes to clot - 10-13 seconds. looks at extrinsic and common pathways (in APTT add contact factor (silica/kaolin) to partial thromboplastin (phospholipids) - 26-38s)

When does haem synthesis take place in a RBC

Between the pro-erythroblast to reticulocyte stage | Hb- 3 coding axons and 2 intervening introns

What are some examples causing warm type (IgG) haemolytic anaemia (AI)?

Idiopathic, drug induced - Hapten, lymphoproliferative disorders (NHL/CLL) (spherocytic and polychromatic)

Give some features and examples of severe inherited platelet disorders.

Mucosal type bleeding pattern, AR Bernard soulier syndrome - absent/defective GP iB/ V/ IX, macrothrombocytopenia Glansmann thrombasthenia - Absent/defective GP IIb/IIIa, normal platelet count

What is characteristic of MDS?

Increased myeloblast cells | Progressive bone marrow failure

What is the IPSS risk score in MDS based off?

% blast cells in BM Karyotype Cytopenia

What is Thalassaemia?

Inadequate production/ decreased synthesis of normal globulin. Relative lack of globulin genes

What cells may be seen in a peripheral smear in sickle cell disease?

Howell bodies

What are some consequences of sickle cell disease in other organs?

Stroke, acute chest syndrome, pulmonary hypertension, vascular retinopathy, kidney infarction, osteonecrosis

what antigen characterises a leukaemia cell?

CD34

what is the main cell produced in acute leukaemia?

Blast cells

What are some clinical signs in Acute leukaemia?

Lethargy, infection, bleeding and bruising, bone pain, gum swelling, lymphadenopathy. Rapid onset

What chemo agents may be used in AML

Anthracycline and cytarabine based

What are the translocation in M3 and M2 AML?

M3 - t(15, 17) - Acute Promyeloblastic leukaemia | M2 - t(8, 21)

What antigens are expressed in CLL?

CD5, CD19, CD23

What are some immune complications of CLL?

AI haemolytic anaemia | AI thrombocytopenia

What is 17p deletion?

In CLL Upper part of Ch17 is missing which allows the cell to recognise and eliminate cells that don't make the quality grade. Loss of P53

How might someone with plasma cell myeloma present?

Recurrent infection, back or rib pain, fatigue, hypercalcaemia, renal impairment

What is the diagnosis criteria for plasma cell myeloma?

Neoplastic plasma cells in BM >/= 10% of total cells plus at least one of: 1. Evidence of end-organ damage attributable to plasma cell proliferation - CRAB (Hypercalcaemia, renal insufficiency, anaemia, Lytic bone lesions) 2. Biomarkers of malignancy - Clonal plasma cell % >/= 60%, serum free light chain ratio, focal lesion on MRI)

What is follicular lymphoma at the molecular level?

t(14,18), over expression of BCL2, slow growth but reduced apoptosis

What immunophenotype is associated with chronic Lymphocytic leukaemia?

CD 5, 19, 23

What may cause purpuric rash with low platelets in the absence of any other cause?

Immune thrombocytopenia purpura

Why shouldn't you give amoxicillin in infectious mononucleosis?

Causes a rash

What might you see in GvHD?

Skin rash, jaundice, diarrhoea

What cells might you see a lot of in MDS?

Myeloblasts

What does sideroblastic anaemia mean?

Failure of Iron to be incorporated into Haem | Hypochromic microcytic

What causes B12 deficiency?

Pernicious anaemia PPi, metformin, contraceptive pills Terminal ileal problems - crohns

Give examples of severe inherited platelet disorders and what it means?

AR. mucosal type bleeding pattern Bernard soulier syndrome Glansmann thrombasthenia

What is the most common Leukaemia?

Chronic lymphocytic leukaemia | Its incidence rises with age

What Tx would you give in essential thermbocytosis?

Hydroxycarbamide + aspirin Anagredlide + aspirin Ruxolitinib INF- alpha