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Pathology - Y5 > Haematological Changes in Systemic Disease > Flashcards

Haematological Changes in Systemic Disease Flashcards

1
Q

What can cause haematological disorders?

A

Haemostasis & Thrombophilia: Altered function of soluble proteins e.g. FVIII deficiency, Protein C deficiency, Platelet function & interaction with endothelial surfaces

Haematological oncology: Primary abnormalities of white cell production
and differentiation, leukaemia lymphoma myeloma

Red cell disorders: Inherited (sickle cell, thalassaemia) acquired

Immune haematology: Autoimmune cytopenias

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2
Q

What can deficiencies cause?

A

Factor VIII: Haemophilia A
Factor IX: Haemophilia B

Protein C: Pro-thrombotic

Erythrocytes: anaemia

Lymphocytes: Lymphopenia (HIV)

Platelets: ITP

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3
Q

What can excesses cause?

A

Erythrocytes: Polycythaemia

Granulocytes: Leukaemia (CML), reactive eosinophilia

Lymphocytes: Leukaemia(CLL)

Platelets: Essential thrombocythemia

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4
Q

What are primary haematological disorders?

A

Primary disorders arise from DNA mutation(s)

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5
Q

What are some examples of primary haematological disorders?

A

Germline/inherited (gene mutated)

  • FIX
  • Erythrocytes e.g. beta Thalassaemia or Chuvash polycythaemia

Somatic/acquired (gene mutated)

  • Erythrocytes e.g. JAK2 V617F: Polycythaemia vera
  • Myeloid/granulocytes e.g. BCR-ABL1: Chroinic myeloid leukaemia
  • Soluble factors
  • No acquired (somatic) DNA mutations, not rapidly dividing cells (hepatocytes /endothelial cells)
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6
Q

What are secondary haematological disorders?

A

Secondary disorders are changes in haematological parameters secondary to a non-haematological disease

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7
Q

What are some examples of secondary haematological disorders?

A

Erythrocytes

  • Exces: cyanotic heart disease
  • Deficiency: anti-RBC antibodies: Immune haemolysis

Factor VIII

  • Excess: inflammatory response
  • Deficiency: anti-FVIII auto-antibodies (acquired haemophilia A)
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8
Q

What can cause increased FVIII?

A

Inflammation

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9
Q

What can cause increased or decreased erythrocytes?

A

Raised - Altitude/hypoxia or Epo secreting tumour

Reduced - BM infiltration or deficiency disease (Vit B12 or Fe)
Shortened survival (Haemolytic anaemia)
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10
Q

What can cause increased or decreased platelets?

A

Raised - Bleeding, Inflammation, splenectomy

Reduced - BM infiltration or deficiency disease (Vit B12)
Shortened survival (ITP, TTP)
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11
Q

What can cause increased or decreased leucocytes?

A

Raised - Infection, Inflammation, corticosteroids

Reduced - BM infiltration or deficiency disease (Vit B12)

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12
Q

What is the cause of iron deficiency unless proven otherwise?

A

Bleeding

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13
Q

What are some reasons for occult blood loss?

A

GI cancers: Gastric, Colonic/rectal

Urinary tract cancers: Renal cell carcinoma, Bladder cancer

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14
Q

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

A

Variable degree of anaemia

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

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15
Q

What are causes of leucoerythroblastic film?

A

Malignant
Haemopoietic: leukaemia/lymphoma/myeloma
Non Haemopoietic: metastatic-breast/bronchus/prostate

Myelofibrosis
Massive splenomegaly
Dry tap on BM aspirate

Severe infection
Miliary TB
Severe fungal infection

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16
Q

What are common laboratory features of all haemolytic anaemias?

A

Anaemia (though may be compensated)

Reticulocytosis

Unconjugated bilirubin raised (pre-hepatic)

LDH raised

Haptoglobins reduced

17
Q

What are the two groups of haemolytic anaemia?

A

Primary (inherited)

Secondary (acquired)

18
Q

What are the features of primary inherited haemolytic anaemia?

A

Defects of the red cell/Germline DNA mutation.

Membrane: e.g. Hereditary Spherocytosis

Cytoplasm/enzymes: e.g. G6PD deficiency

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

19
Q

What are the features of secondary acquired haemolytic anaemia?

A

Defects of the environment (systemic disease) in which the red cell finds itself:

Non Immune (DAT –ve)

Immune mediated (Direct Antiglobulin Test (DAT) (aka Coombs
test) +ve)
20
Q

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

A

Spherocytes

Associated with systemic diseases involving Immune system

  • Malignancy: e.g. Lymphoma or CLL
  • Autoimmune: e.g. SLE
  • Infection: e.g. mycoplasma
  • Idiopathic
21
Q

What is associated with DAT -ve haemolytic anaemia?

A

Infection: Malaria

Micro-angiopathic Haemolytic anaemia (MAHA)

  • Underlying adenocarcinoma
  • Haemolytic uraemic syndrome
22
Q

What is microangiopathy associated with and what happens?

A

Adenocarcinomas, low grade DIC

  • Platelet activation
  • Fibrin deposition and degradation
  • Red cell fragmentation (microangiopathy)
  • Bleeding (low platelets and coag factor deficiency)

USUALLY associated with malignancy

23
Q

What are some causes of neutrophilia?

A

Corticosteroids

Underlying neoplasia

Tissue inflammation (e.g.colitis, pancreatitis)

Myeloproliferative/leukaemic disorders

PYOGENIC INFECTION

24
Q

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

A

Neutrophilia + toxic granulation no immature cells

25
Q

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

A

Neutrophilia plus basophilia & immature cells myelocytes

26
Q

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

A

Neutropenia plus myeloblasts suggests acute leukaemia (AML)

27
Q

What are some causes of eosinophilia?

A

Reactive eosinophilia
- Parasitic infestation
Allergic diseases e.g. asthma, rheumatoid, polyarteritis,pulmonary eosinophilia.
Underlying Neoplasms, esp. Hodgkin’s, T-cell NHL (reactive eosinophilia)
Drugs (reaction erythema multiforme)

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

28
Q

What is monocytosis?

A

Rare but seen in certain chronic infections and primary haematological disorders.

  • TB, brucella, typhoid
  • Viral; CMV, varicella zoster
  • Sarcoidosis
  • Chronic myelomonocytic leukaemia (MDS)
29
Q

What are some causes of increased lymphocytes?

A

EBV, CMV, Toxoplasma

Infectious hepatitis, rubella, herpes infections

Autoimmune disorders

Sarcoidosis

30
Q

What are some causes of decreased lymphocytes?

A

Infection HIV

Auto immune disorders

Inherited immune deficiency syndromes

Drugs (chemotherapy)

31
Q

What are differentials for unusual blood films with lymphocytosis?

A

Mature lymphocytes (PB)

  • Reactive/atypical lymphocytes (IM)
  • Small lymphocytes and smear cells (CLL/NHL)

Immature Lymphoid cells in PB
- Lymphoblasts (Acute Lymphoblastic Leukaemia)

32
Q

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

A

If it is polyclonal (Kappa and Lambda chain diversity) then it is reactive, if it is monoclonal, then it is malignant.

33
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)

34
Q

What are examples of cellular proliferation?

A

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

  • BCR-ABL1 CML
  • JAK2 MPD
35
Q

What are examples of impaired/blocked cellular differentiation?

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

36
Q

What are examples of prolonged cell survival?

A

Mutations in apoptosis genes may occur in lymphomas

  • BCL2 and Follicular lymphoma

Pathology - Y5 (59 decks)

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