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Path Y > Haematology - Haematology in Systemic Disease > Flashcards

Haematology - Haematology in Systemic Disease Flashcards

1
Q

<p>What disease is characterised by primary raised erythrocytes?</p>

A

<p>Polycythaemia vera</p>

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2
Q

<p>What disease is characterised by an acquired reduction in erythrocytes?</p>

A

<p>Auto-immune haemolytic anaemia</p>

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3
Q

<p>Which disease is caused by a genetic deficiency of factor IX?</p>

A

<p>Haemophilia B</p>

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4
Q

<p>Which disease is caused by a genetic excess of factor IX?</p>

A

<p>Factor IX Padua</p>

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5
Q

<p>Which disease is caused by a genetic deficiency of erythrocytes?</p>

A

<p>Sickle cell disease/ HbS (beta globin gene mutation)</p>

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6
Q

<p>How can haemophilia B be treated using gene therapy?</p>

A

<p>Factor IX Padua gene can be put into adenoviruses as a vector, to cause factor IX production</p>

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7
Q

<p>Which disease is caused by an acquired mutation in JAK2?</p>

A

<p>Polycythaemia vera</p>

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8
Q

<p>Which disease is caused by an acquired mutation in PIG A?</p>

A

<p>PNH paroxysmal nocturnal haemoglobinuria<br></br>PIG A = phosphatidylinositol glycan A</p>

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9
Q

<p>What is a raised factor VIII likely to be secondary to?</p>

A

<p>An inflammatory process</p>

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10
Q

<p>How can haemophilia be acquired? (Rather than genetic)</p>

A

<p>Auto-immune disorder common in elderly - body produces autoantibodies directed against factor VIII</p>

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11
Q

<p>Recall 2 secondary causes of raised erythrocytes?</p>

A

<p>Altitude
<br></br>EPO-secreting tumour</p>

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12
Q

<p>Recall 3 secondary causes of reduced erythrocytes</p>

A

<p>Bone marrow inflitration
<br></br>Deficiency (B12/Fe)
<br></br>Haemolytic anaemia</p>

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13
Q

<p>What is the most likely cause of iron deficiency anaemia?</p>

A

<p>Bleeding (until proven otherwise!)</p>

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14
Q

<p>How is iron deficiency anaemia diagnosed?</p>

A

<p>Ferritin and transferrin saturation: both would be low</p>

TIBC high

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15
Q

<p>Recall 3 types of cancer that may present first with an iron deficiency anaemia?</p>

A

<p>Gastric
<br></br>Renal
<br></br>Bladder</p>

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16
Q

<p>What are 3 morphological features of leuco-erythroblastic anaemia?</p>

A

<p>Teardrop RBCs (+aniso and poikilocytosis)  <br></br>Nucleated RBCs (erythroblasts)<br></br> Immature myeloid cells</p>

note nucleated RBCs are normal in BM, abnormal in PB

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17
Q

<p>What are 3 causes of leucoerythroblastic anaemia?</p>

A

<p>Infection: miliary TB/severe fungal infection<br></br>Malignancy: leukaemia/lymphoma/myeloma/ metastatic Ca<br></br>Myelofibrosis- dry tap on BM aspirate <br></br>USUALLY A MALIGNANCY INVOLVING BM</p>

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18
Q

<p>What are 5 laboratory features of haemolytic anaemias?</p>

A 
<p>Anaemia (though may be compensated)
<br>
<br>Reticulocytosis
<br>
<br>Unconjugated hyperbilirubinaemia
<br>
<br>LDH raised 
<br>
<br>Reduced haptoglobins</p>
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19
Q

<p>Which test detects auto immune haemolytic anaemia?</p>

A

<p>DAT/Coombs</p>

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20
Q

<p>Recall 2 malignancies that can cause a warm auto immune haemolytic anaemia?</p>

A

<p>Lymphoma

| <br></br>CLL</p>

21
Q

<p>What type of anaemia is caused by mycoplasma infection?</p>

A

<p>cold autoimmune haemolytic anaemia</p>

22
Q

<p>What are 3 causes of non-immune/ Dat neg haemolytic anaemia?</p>

A

<p>1. Malaria
<br></br>2. MAHA - microangiopathic haemolytic anaemia</p>

  1. o Paroxysmal nocturnal haemoglobinuria
23
Q

<p>Recall 3 causes of MAHA</p>

A

<p>1. Underlying adenocarcinoma<br></br>2. Haemolytic uraemic syndrome</p>

  1. TTP
24
Q

<p>How can CLL and AML be distinguished on blood film?</p>

A

<p>CLL has mature cells, AML has immature cells</p>

Blasts, which are immature and dysfunctional cells, normally make up 1% to 5% of marrow cells. Acute leukemias are characterized by greater than 20% blasts in the peripheral blood smear or on bone marrow leading to a more rapid onset of symptoms. In contrast, chronic leukemia has less than 20% blasts with a relatively chronic onset of symptoms.

25
Q

<p>What is the main cause of neutrophilia?</p>

A

<p>Pyogenic infection</p>

other cause - corticosteroids

26
Q

<p>How do you distinguish a reactive/infective neutrophilia vs a malignant one?</p>

A

<br></br>No immature cells in reactive neutrophilia
<br></br>
<br></br>Malignant has
immature cells + either basophils (indicative of CML) or myeloblasts (indicative of AML)
in malignant, neutrophils will be massively raised

27
Q

<p>What is the most common cause of a reactive lymphopaenia?</p>

A

<p>HIV</p>

28
Q

<p>In which 2 conditions might smear/smudge cells appear on the blood film?</p>

A

<p>CLL<br></br>Non-Hodgkins lymphoma</p>

29
Q

<p>Name 4 causes of an inherited haemolytic anaemia</p>

specify the defects.

A

<p>Any of: <br></br>Hereditary spherocytosis - membrane
<br></br>G6PD deficiency - enzyme
<br></br>Sickle cell haemoglobin structural
<br></br>Thalassaemia - haemoglobin quantitative </p>

30
Q

<p>How can DIC cause MAHA?</p>

A

maha = • Deposition of platelets in small blood vessels

disseminated intravascular coagulopathy may cause a microangiopathic hemolytic anemia because of fibrin deposition that result from damage to small blood vessels.

31
Q

<p>How is B cell clonality determined?</p>

A

<p>Look for light chain restriction - the ratio of kappa and lambda (60:40 would be reactive, 99:1 would be malignant)</p>

The detection of immunoglobulin light chain restricted B cell population is considered a surrogate marker of clonality, which can be confirmed by molecular assays. In general, the presence of a monotypic B cell population in the ascitic fluid is considered lymphomatous involvement rather than a reactive condition.

32
Q

<p>Recall 2 tests that can determine immunophenotype in blood malignancies, and one use of knowing this info?</p>

A

Immunophenotyping – best way to determine lymphoid from myeloid – will show antigens

 Flow cytometry (antibodies tagged to fluorescent antibody)
 Immunocytochemistry (monoclonal antibodies added)

33
Q

<p>Recall 2 tests that can determine cytogenetics in blood malignancies and one use of knowing this info?</p>

A

FISH
PCR

can identify translocations
e.g t (11; 14) = mantle cell lymphoma

34
Q

<p>Recall 2 tests that can determine molecular genetics in blood malignancies and one use of knowing this info?</p>

A

<p>PCR
<br></br>Pyro sequencing
<br></br>Use: Detect JAK2 mutation in PV, or BCR ABL in CML cDNA</p>

35
Q

what condition is the ham test sued to identify?

A

The Ham test is a blood test to diagnose paroxysmal nocturnal hemoglobinuria (PNH). This rare condition causes red blood cells to die before they should. The Ham test checks whether red blood cells become more fragile when they are placed in mild acid.

36
Q

what’s the pentad of TTP

A

haemolytic anaemia, thrombocytopenia, AKI, neurological impairment, fever

37
Q

what’s the triad of HUS

A

MAHA, thrombocytopenia, AKI

38
Q

compare warm and cold AIHA? which is more common

A 
Warm AIHA (80-90%)
Warm antibody hemolytic anemia can often be differentiated from cold agglutinin disease by the temperature at which the direct antiglobulin test is positive; a test that is positive at temperatures ≥ 37° C indicates warm antibody hemolytic anemia, whereas a test that is positive at lower temperatures indicates cold .
39
Q

summarise the types of haemolytic anaemia

A

inherited - SCD, thal, G6PDD, hereditary speherocytosis

acquired - immune (warm & cold) and non-immune (malaria, MAHA, PHN)

40
Q

negative Coombs & spheroctyes =

A

hereditary spherocytosis

41
Q

postive Coombs (DAT+ve) and spheroctyes =

A

AIHA

42
Q

when do you see reactive eosinophilia (4)

A

 Parasitic infection
 Allergic diseases  asthma, rheumatoid, polyarteritis, pulmonary eosinophilia
 Underlying neoplasms  Hodgkin’s, T-cell NHL
 Drugs = reaction erythema multiforme

43
Q

2 causes of lymphopenia

A

HIV, chemo

44
Q

2 causes of lymphocytosis

A

sarcoid, EBV, CMV, Toxoplasma

 Infectious hepatitis, rubella, herpes infections

45
Q

mutations in tyrosine kinase generally cause what? give 2 specific examples

A 
cellular proliferation (no impact on differentiation)
	BCR-ABL = CML
	JAK2 = MPD
46
Q

mutations in nuclear transcription factors (NF-Y eg) generally cause what? give a specific example

A

they block differentiation

 PML RARA fusion gene in APL

47
Q

mutations in apoptosis genes generally occur in what cancer what? give an example

A

generally occur in lymphomas

 BCL2 gene = follicular lymphoma

48
Q

give 4 ways to study malignant cells

A

 Morphology Immunophenotype

 Cytogenetics Molecular Genetics

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