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YR5 Haematology > Haematological Changes in Systemic Disease > Flashcards

Haematological Changes in Systemic Disease Flashcards

1
Q

What haematological manifestations arise from altered function of soluble proteins?

A

Haemostasis: FVIII deficiency = bleeding tendency

Thrombophilia: Protein C deficiency = thrombotic tendency

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2
Q

What haematological disorders arise from abnormalities of cell number/ function?

A

WBC: Leukaemia, reactive leukocytosis, myeloma
RBC: Sickle cell, Thalassaemia

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3
Q

What are the other broad etiological groups of haematological disorders?

A

Immune: AI disorders

Transfusion associated

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4
Q

What conditions are caused by haematological deficiencies?

Factor VIII, IX, Protein C, Erythrocytes, Lymphocytes, Platelets

A

Factor VIII: Haemophilia A
Factor IX: Haemophilia B
Protein C: Pro-thrombotic
Erythrocytes: anaemia
Lymphocytes: Lymphopenia (HIV)
Platelets: ITP

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5
Q

What conditions are caused by haematological excesses?

Erythrocytes
Granulocytes
Lymphocytes
Platelets

A

Erythrocytes: Polycythaemia

Granulocytes: Leukaemia (CML), reactive eosinophilia

Lymphocytes: Leukaemia (CLL)

Platelets: Essential thrombocythemia

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6
Q

What are primary haematological disorders?

A

Inherited (germline gene mutated) or acquired (somatic gene mutated) DNA mutation(s)

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7
Q

What are most haematological malignancies due to?

A

Acquired (somatic) gene mutations

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8
Q

Give a primary and secondary condition causing raised erythrocytes

A

Primary: Polycythaemia Vera

Secondary: High altitude

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9
Q

Name 2 inherited mutations of FIX and erythrocytes that cause primary haematological disorders

A

FIX
Deficiency: Haemophilia B (bleeding)
Excess: FIX Padua (thrombosis)

Erythrocytes
Deficiency: Hb S
Excess: Polycythaemia

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10
Q

Name 2 ACQUIRED haematological mutations of erythrocytes that cause primary disorders

A

JAK2 V617F: Polycythaemia vera

PIG A: PNH Paroxysmal nocturnal haemoglobinuria

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11
Q

Why are there no acquired DNA mutations in soluble factors to cause primary haematological disorder?

A

Soluble factors are secreted by endothelial cells or hepatocytes

Not by rapidly dividing cells which have greater risk of mutation

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12
Q

Give a primary and secondary disorder causing reduced erythrocytes

A

Primary: Thalassaemia

Secondary: AI haemolytic anaemia

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13
Q

What are secondary haematological disorders?

A

Changes in haematological parameters in response to a non-haematological disease/ condition

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14
Q

What are the FVIII examples of secondary haematological disorders?

A

Excess: inflammatory response/ pregnancy
Deficiency: anti-FVIII auto-antibodies (acquired haemophilia A)

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15
Q

What systemic condition can cause increased FVIII? What is the consequence of this?

A

Chronic inflammation

Increases thrombosis risk

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16
Q

What systemic changes can cause increased or decreased erythrocytes?

A

Increased: Altitude/ hypoxia or EPO secreting tumour

Decreased: BM infiltration or deficiency disease (Vit B12 or Fe)
Shortened survival (Haemolytic anaemia)

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17
Q

What systemic changes can cause increased or decreased platelets?

A

Increased: Bleeding, Inflammation, splenectomy

Decreased: BM infiltration or deficiency disease (Vit B12)
Shortened survival (ITP, TTP)

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18
Q

What systemic changes can cause increased or decreased leucocytes?

A

Increased: Infection, Inflammation, corticosteroids

Decreased: BM infiltration or deficiency disease (Vit B12)

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19
Q

What systemic disease would you associate with folate deficiency, macrocytic anaemia and Howell Jolly bodies?

A

Coeliac

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20
Q

Give 2 examples of anomalous blood results that may be the first presentation of malignancy

A

Microcytic anaemia: GI cancer

Hypercalcaemia: Myeloma

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21
Q

What is the cause of iron deficiency unless proven otherwise?

A

Bleeding

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22
Q

What are the labratory findings in iron deficiency?

A

Microcytic hypochromic anaemia

Low ferritin

Low transferrin saturation

High TIBC

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23
Q

What are 2 classes of reasons for occult blood loss?

A

GI:
Peptic ulcer or Gastric cancer
IBD or Colonic cancer

Urinary tract:
Renal cell carcinoma
Bladder cancer

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24
Q

What is Leuco-erythroblastic anaemia and what are the signs in a blood film?

A

Variable degree of anaemia (mild-severe)

Teardrop RBCs (+aniso + poikilocytosis)
Nucleated RBCs
Immature myeloid cells

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25
Q

Why is leuco-erythroblastic anaemia an important finding? What can cause this?

A

It implies abnormal BM infiltration

Malignancy
Myelofibrosis
Severe infection (rarely)

26
Q

What are the malignant causes of a leuco-erythroblastic anaemia?

A

Haematopoietic: Leukaemia, Lymphoma, Myeloma

Metastatic: Breast, Bronchus, Prostate

27
Q

What features would indicate myelofibrosis being causative of a leuco-erythroblastic anaemia?

A

Massive splenomegaly

Dry tap on BM aspirate

28
Q

Which infections could cause a leuco-erythroblastic anaemia?

A

Miliary TB

Severe fungal infection

29
Q

What are common laboratory features of all haemolytic anaemias?

A

Anaemia (though may be compensated)

Reticulocytosis (high MCV)

HIGH Unconjugated BR (pre-hepatic, excess BR)

HIGH LDH (intracellular enzyme released)

LOW Haptoglobins

30
Q

What are the two groups of haemolytic anaemia?

A

Primary (inherited)

Secondary (acquired)

31
Q

What are the features of primary inherited haemolytic anaemia?

A

Inherited mutation of RBC

Membrane: Hereditary Spherocytosis

Cytoplasm/ enzymes: G6PD deficiency

Haemoglobin: Sickle cell disease (structural); Thalassaemia (quantitative)

32
Q

What are the features of secondary acquired haemolytic anaemia?

A

Defects of RBCs environment (systemic disease):

Non Immune (DAT –ve)

Immune mediated (DAT +ve)

Direct Antiglobulin Test = DAT (aka Coombs test)

33
Q

What are the features of DAT +ve haemolytic anaemia and what is it associated with?

A

Spherocytes

A/W systemic diseases
Malignancy: Lymphoma or CLL
AI: SLE
Infection: mycoplasma
Idiopathic

34
Q

What is associated with DAT -ve haemolytic anaemia?

A

Infection: Malaria

Micro-angiopathic Haemolytic anaemia (MAHA): indicates underlying adenocarcinoma
or Haemolytic uraemic syndrome

35
Q

What is microangiopathy associated with and what happens?

A

Adenocarcinomas, low grade DIC

  1. Platelet activation
  2. Fibrin deposition + degradation
  3. RBC fragmentation (microangiopathy, BP drives RBC through fibrin strands)
  4. Bleeding (low platelets + coag factor deficiency)

USUALLY a/w malignancy

36
Q

What are 5 causes of neutrophilia?

A

PYOGENIC INFECTION

Corticosteroids

Underlying neoplasia

Tissue inflammation (e.g.colitis, pancreatitis, MI)

Myeloproliferative/ leukaemic disorders

37
Q

What would you expect to see in a blood film for an infection?

A

Neutrophilia + toxic granulation no immature cells

38
Q

What would you expect to see in a blood film for CML?

A

Neutrophilia PLUS Basophilia + Myelocytes

39
Q

What would you expect to see in a blood film for AML?

A

Neutropenia plus Myeloblasts

40
Q

4 causes of reactive eosinophilia?

A

Parasitic infestation
Allergic diseases: asthma, RA, polyarteritis, pulmonary eosinophilia.
Underlying Neoplasms, esp. Hodgkin’s, T-cell NHL
Drugs (reaction erythema multiforme)

41
Q

What chronic infections and primary haematological disorders cause monocytosis?

A

RARE

TB, brucella, typhoid
CMV, VZV
Sarcoidosis
Chronic myelomonocytic leukaemia (MDS)

42
Q

What are 4 causes of lymphocytosis?

A

EBV, CMV, Toxoplasma

Infectious hepatitis, rubella, herpes infections

AI disorders

Sarcoidosis

43
Q

What are 4 causes lymphopenia?

A

Infection: HIV

AI disorders

Inherited immune deficiency syndromes

Drugs (chemotherapy)

44
Q

What are the differentials for lymphocytosis with mature lymphocytes in the peripheral blood?

A

Reactive/ atypical lymphocytes: IM
Small lymphocytes + smear cells: CLL/ NHL

45
Q

How do you determine if B-cell lymphocytosis is malignant or reactive?

A

Reactive: Polyclonal (Kappa + Lambda chain diversity, 60:40)

Malignant: Monoclonal (Kappa OR Lambda only, 99:1)

46
Q

What are the three types of acquired somatic mutations?

A

Cellular proliferation (Type 1)

Impair/ block cellular differentiation (Type 2)

Prolong cell survival (Anti-apoptosis)

47
Q

How do cellular proliferation mutations cause malignancy? Give 2 examples

A

Mutations in Tyrosine Kinase genes cause excess proliferation (no effect on differentiation)

BCR-ABL: CML

JAK2: MPD

48
Q

How do mutations causing impaired/ blocked cellular differentiation lead to malignancy? Give an example

A

Mutations in nuclear transcription factors may block differentiation. If present along with a proliferation mutation can cause acute leukaemia

PML-RARA in acute promyelocytic leukaemia

49
Q

In which malignancies are there mutations that cause prolonged cell survival?

A

Mutations in apoptosis genes may occur in lymphomas

BCL2 + Follicular lymphoma

50
Q

Which white cells are found in the bone marrow?

A

Myeloblasts

Lymphoblasts

Promyelocytes

Myelocytes

51
Q

Which white cells are found in peripheral blood?

A

Phagocytes: Granulocytes + Monocytes

Immunocytes: T + B lymphocytes, NK cells

52
Q

List 3 types of granulocyte

A

Neutrophils

Eosinophils

Basophils

53
Q

What malignancy causes secondary eosinophilia?

A

Chronic eosinophilic leukaemia
Eosinophils part of the “clone”
FIP1L1-PDGFRa Fusion gene

54
Q

What is the differential for lymphocytosis with immature lymphoid cells in the peripheral blood (PB)?

A

Acute Lymphoblastic Leukaemia

55
Q

What investigations are performed on a tissue biopsy to establish haematological malignancy diagnosis?

A
  1. Morphology: architecture
  2. Immunophenotype: flow cytometry or immunohistology
  3. Cytogenetics: Translocations- fusion gene or de-regulated oncogene
  4. Molecular genetics: PCR
56
Q

What is determined in immunophenotyping?

A

Myeloid or Lymphoid

T or B lineage

Stage of maturation

57
Q

How does lympho-haemopoietic failure manifest and present in patients?

A

BM (Myeloid): Anaemia, Bacterial infection (neutrophils), Bleeding (platelets)

Immune system (Lymphoid): Recurrent viral or fungal infection

58
Q

How does excess of malignant cells manifest and present in patients?

A

Erythrocytes/ Leukocytes: Impair blood flow- Stroke

Massively enlarged LN-lymphoma: compress hollow tubes- bowel, vena cava, uterus, bronchus

59
Q

Which organs may be infiltrated and impaired in haematological malignancy?

A

CNS lymphoma

Skin lymphoma

Kidney failure (light chain deposition from myeloma)

60
Q

Give 2 miscellaneous problems caused by haematological malignancy

A

Hypercalcaemia

Hypermetabolism

YR5 Haematology (20 decks)

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