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Haematology > Haem: Haem changes in systemic diseases and intro to haemopath > Flashcards

Haem: Haem changes in systemic diseases and intro to haemopath Flashcards

1
Q

What is it called when there is a def in Factor 8

A

Haemophilia A

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2
Q

What is it called when there is an excess of Hb

A

Polycythaemia - Due to hypoxia or EPO secreting tumour

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3
Q

What is it called when there is an excess of granulocytes

A

CML or reactive eosinophilia

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4
Q

What is it called when there is an excess of lymphocytes

A

CLL

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5
Q

What is it called when there is a def of lymphocytes

A

Lymphopenia (HIV)

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6
Q

What is it called when there is an excess of platelets

A

Essential thrombocythemia

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7
Q

What is it called when there is a def of platelets

A

ITP

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8
Q

What can cause anaemia?

A

Vit B12 or iron def

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9
Q

What is the difference between primary and secondary haem disorders?

A

Primary - Germline or somatic mutations affecting bone marrow
Secondary - Changes in haem parameters secondary to a non haem disease

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10
Q

What are some germline mutations in haem?

A

Factor 9 def - Haemophilia B
B chain globulin chain production defect - B thalaseeaemia
VHL gene mutation in RBC - Chuvash polycythaemia

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11
Q

What are some somatic mutations in haem?

A

JAK2 V617F mutation in RBC - Polycythaemia vera

BCR-ABL1 in myeloid granulocytes - Chronic myeloid leukaemia

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12
Q

What are some examples of secondary haem disorders?

A

Immune haemolysis - Anti RBC antibodies

Anti-factor 8 auto antibodies - acquired haemophilia A

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13
Q

What would the lab results for Fe def anaemia be?

A

Microcytic hypochromic
Reduced ferretin and transferrin saturation
Raised TIBC

The cause is always bleeding until proven otherwise

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14
Q

What is leuco erythroblastic anaemia and what would the blood film results look like?

A

Precursor to RBC and WBC anaemia - variable degrees of anaemia

Teardrop RBC - abnormal size and shape of RBCs
Nucleated RBC
Immature myeloid cells

RBCs are supposed to lose their nucleus before leaving the bone marrow, so this shows that there is some level fo damage in the bone marrow

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15
Q

What are the reasons for bone marrow infiltration

A

Malignant - Leukaemia, lymphoma, myeloma, metastatic cancers
Infection - Miliary TB, severe fungal infection
Myelofibrosis - Massive splenomegaly, dry tap on BM aspirate

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16
Q

What are some common features of haemolytic anaemia?

A

Reticulocytes
Raised unconjugated bilirubin
LDH raised
Haptoglobins lowered

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17
Q

What could be cause of haemolytic anaemia?

A

Sickle cell, malaria, auto immune, G6PD

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18
Q

What are the 2 groups of haemolytic anaemia?

A

Inherited and acquired

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19
Q

What are some causes of inherited haemolytic anaemia?

A

Membrane defect - Hereditary spherocytosis
Cytoplasm/Enzyme defect - G6PD deficiency
Hb defect - Sickle cell disease, Thalassemia

20
Q

What are the 2 types of acquired haemolytic anaemia?

A

Immune - DAT/coombs test positive

Non-immune

21
Q

What would you see in autoimmune haemolytic anaemia

A

Spherocytes

DAT +ve

22
Q

What are some associated diseases with autoimmune acquired haemolytic anaemia

A

Lymphoma, CLL, SLE, mycoplasma

23
Q

What are some causes of non-immune acquired haemolytic anaemia

A

Malaria

MAHA

24
Q

What is particularly concerning about MAHA

A

It could be due to underlying adenocarcinoma or haemolytic uraemic syndrome in children due to E coli infection

25
Q

What would you see on MAHA blood film?

A

RBC fragments

Thrombocytopaenia

26
Q

When would you see spherocytosis?

A

Inherited or autoimmune haemolytic anaemia

27
Q

How would any malignant metasteses to the the bone marrow present?

A

Low Hb, low reituculocytes, high conjugated bilirubin

28
Q

What can cause neutrophilia?

A 
Underlying neoplasm
Corticosteroids 
Inflammation
Myeloproliferative/Leukaemic disorders 
Pyogenic infections (pus forming bacteria)
29
Q

When there is neutrophilia, what are the 2 causes?

A

Infection/ Reactive or Malignant

30
Q

What would you see in neutrophils in an infection?

A

No immature cells
Raised neutrophils
Toxic granulation

31
Q

What would you see in neutrophils in CML?

A

Neutrophilia + basophils + Immature myelocytes

32
Q

What would you see in neutrophils in AML?

A

Neutrophilia + Myeloblasts

33
Q

What can cause reactive eosinophilia?

A

Parasitic infection
Allergic diseases
Underlying neoplasms - Hodgkin’s, T cell NHL
Drug reactions (erythema multiforme)

34
Q

What do pox viruses generally cause

A

Basophilia

35
Q

What happens in chronic eosinophillic leukaemia

A

FIP1L1-PDFGRa fusion gene

36
Q

What can cause monocytosis

A

TB, Brucella, typhoid
CMV, varicella zoster
sarcoidosis
Chronic myelomonocytic leukaemia

37
Q

What can cause lymphocytosis

A

EBV, CMV, toxoplasma, hepatitis, rubella, herpes,sarcoidosis

38
Q

What can cause lymphopaenia

A

HIV, chemo

39
Q

When can you see mature lymphocytes in blood

A

Glandular fever, CLL, NHL - small lymphocytes and smear cells

40
Q

When can you see immature lymphocytes / lymphoblasts

A

ALL

41
Q

How do you differentiate between healthy/reactive polyclonal lymphocytosis and monoclonal lymphocytosis

A

Healthy/reactive - 60:40 split kappa and lambda expressing cells
Monoclonal - 90% either kappa or lamda - malignancy

42
Q

What are the 4 stages of testing you go through for haem cancers

A

Morphology
Immunophenotype
Cytogenetics
Molecular genetics

43
Q

What happens in myeloproliferative neoplasm (chronic)

A

There is an overproliferation of neutrophils

44
Q

What happens in acute myeloid leukaemia

A

Differentiation of myeloblasts is blocked, so you get an overproliferation of immature cells

45
Q

What are 3 types of acquired somatic mutations in leukaemia and lymphoma

A

Cellular proliferation - Mutation in tyrosine kinase (BCR ABL in CML and JAK2 in MPD)

Blocked cellular differentiation

Prolonged cell survival - BCL 2 and follicular lymphoma

46
Q

How do you differentiate between B cell acute lymphoblastic lymphoma and multiple myeloma

A

B cell acute lymphoblastic lymphoma - Tdt +ve, CD19 +ve, Surface Ig -ve

Multiple myeloma - Tdt +ve, Surface Ig +ve, CD138 -ve

Haematology (10 decks)

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