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Haematology (E) > Haematological Changes in Systemic Disease > Flashcards

Haematological Changes in Systemic Disease Flashcards

1
Q

What are the main 4 types of haematological disorders?

A

Haemostasis and thrombophilia
Haematological oncology
Red cell disorders
Immune haematology

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2
Q

What broadly happens in haemostasis and thrombophilia?

A

Altered function of solube=le proteins e.g. FVIII deficiency, Protein C deficiency
Platelet function & interaction with endothelial surfaces

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3
Q

What are the main causes of haematological oncology?

A

Primary abnormalities of white cell production and differentiation

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4
Q

What are the common red cell disorders?

A

Inherited (sickle cell, thalassaemia) acquired

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5
Q

What are the main immune haematology conditions?

A

Auto immune cytopenias

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6
Q

What is often the fundamental problem in haematological conditions?

A

Excesses or deficiencies

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7
Q

What is the result of a deficiency of Factor VIII?

A

Haemophilia A –> bleeding

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8
Q

What is the result of a deficiency of Protein C?

A

Pro-thrombotic

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9
Q

What is the name of an excess of erythrocytes?

A

Polycythaemia

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10
Q

What is the name of a deficiency of erythrocytes?

A

Anaemia

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11
Q

What could be the cause of excess of granulocytes?

A

Leukaemia (CML)

Reactive eosinophilia

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12
Q

What could be the cause of excess lymphocytes?

A

Leukaemia (CLL)

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13
Q

What could be the cause of lymphocyte deficiency?

A

HIV –> lymphopenia

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14
Q

What is the name for a raised platelet count?

A

Essential thrombocythemia

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15
Q

What is the name of platelet deficiency precipitated by infection?

A

Immune thrombocytopenic purpura (ITP)

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16
Q

Define primary haematological disorders

A

Primary disorders arise from DNA mutations and are diseases of the bone marrow or blood

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17
Q

Define secondary haematological disorders

A

Changes in haematological parameters secondary to a non-haematological disorder

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18
Q

What condition is a FIX deficiency?

A

Haemophilia B

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19
Q

Give examples of primary erythrocyte disorders

A

Deficiency - beta globin chain production –> beta Thalassaemia
Excess –> VHL gene –> Chuvash polycythaemia

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20
Q

Give an example of a primary acquired erythrocyte disorder

A

Excess –> JAK2 V617F –> Polycythaemia vera

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21
Q

Given an example of a primary acquired myeloid/granulocyte disorder

A

BCR-ABL1 –> Chronic myeloid leukaemia

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22
Q

Give an example of a secondary disorder causing excess erythrocytes

A

Cyanotic heart disease –> hypoxia –> excess erythrocytes

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23
Q

Give an example of a secondary disorder leading to decreased erythrocytes

A

Immune haemolysis –> anti-RBC antibodies –> deficiency

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24
Q

Given an example of a secondary disorder that can lead to FVIII excess

A

Inflammatory response –> FVIII excess

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25
Q

Give an example of a secondary disorder that leads to FVIII deficiency

A

anti-FVIII auto-antibodies in acquired haemophilia A –> FVIII deficiency

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26
Q

Fe deficiency is

A

Bleeding until proven otherwise

27
Q

If Fe is found and there is an underlying malignancy, what will the blood tests show?

A

Fe deficiency
Microcytoic hypochronic anaemia
Reduced ferritin, transferrin saturation
Raised total iron binding capacity (TIBC)

28
Q

What may cause occult blood loss?

A

GI cancers - gastric, colorectal

Urinary tract cancers - renal cell carcinoma, bladder cancer

29
Q

What is a leuco-erythroblastic anaemia?

A

Variable degree of anaemia

30
Q

What are the blood film features in leuco-erythroblastic anaemia?

A

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

31
Q

Normal peripheral blood film features

A

No nucleated RBCs

Mature cells

32
Q

Leucoerythroblastic film

A

Tear drop poikilocytes
Nucleated RBCs
Myelocyte

33
Q

What must have happened for a leucoerythroblastic film?

A

Bone marrow infiltration

34
Q

What are the causes of bone marrow infiltration?

A

Sever infection - miliary TB, severe fungal infection
Myelofibrosis - massive splenomegaly, dry tap on BM aspirate
Malignant - Haemopoietic (leukaemic/lymphoma/myeloma), non-haemopoietic (metastatis breast/bronchus/prostate)

35
Q

What are the common laboratory features of all haemolytic anaemias?

A 
Anaemia (though may be compensated)
Reticulocytosis
Unconjugated bilirubin raised (pre-hapatic)
LDH raised
Haptoglobins reduced
36
Q

What are the groups of haemolytic anaemias?

A

Inherited (primary) - defects of the red cell/germline DNA mutation
Acquired (secondary) - defects of the environment in which the red cell finds itself i.e. systemic disease

37
Q

Give examples of primary haemolytic anaemias

A

Membrane e.g. Hereditary Spherocytosis
Cytoplasm/enzymes e.g. G6PD deficiency
Haemoglobin e.g. Sickle cell disease (structural) Thalassaemia (quantitative)

38
Q

What are the types of acquired (secondary) haemolytic anaemias?

A 
Non immune (DAT -ve)
Immune mediated (DAT +ve, direct antiglobulin test i.e. Coombs test)
39
Q

What are the key features of immune haemolytic anaemia?

A

Spherocytes

DAT +ve (Coombs test)

40
Q

What systemic diseases are associated with immune haemolytic anaemia?

A

Malignancy e.g. Lymphoma, CLL
Auto immune e.g. SLE
Infection e.g. mycoplasma
Idiopathic

41
Q

What are some non-immune causes of acquired haemolytic anaemia?

A

Infection e.g. malaria

Micro-angiopathic Haemolytic anaeia (MAHA) - underlying adenocarcinoma, haemolytic uraemic syndrome

42
Q

Micro-angiopathic haemolytic anaemia blood film

A

RBC fragments

Thrombocytopenia

43
Q

Why does MAHA cause RBC fragments?

A

Platelet activation –> fibrin deposition and degradation –> red cell fragmentation (microangiopathy) –> bleeding (low platelets and coag factor deficiency)

44
Q

What types of white blood cells are found in bone marrow?

A

Blasts (myeloid & lymphoid)
Promyelocytes
Myelocytes

45
Q

What white blood cells are found in peripheral blood?

A

Phagocytes: granulocytes - neutrophils, eosinophil, basophils. monocytes
Immunocytes: T lymphocytes, B lymphocytes

46
Q

What are the immediate concerns if immature cells are seen in the peripheral blood?

A

Leukaemia or metastatic cancer invading bone marrow

47
Q

What can cause neutrophilia (raised neutrophil count)?

A 
Pyogenic infection
Corticosteroids
Underlying neoplasia
Tissue inflammation e.g. colitis, pancreatitis
Myeloproliferative/leukaemic disorders
48
Q

How would you identify a reactive/infection neutrophilia?

A

Neutrophilia + toxic granulation no immature cells

49
Q

How would you identify a malignant neutrophilia?

A

Neutrophilia plus basophilia & immature cells myelocytes. Suggest a myeloproliferative (CML)

Neutrophenia plus myeloblasts suggests acute leukaemia (AML)

50
Q

Reactive neutrophilia blood film

A

Only mature cells, toxic granules in neutrophils

51
Q

CML blood film

A

Neutrophilia
Immature cells (myelocytes)
Basophils

52
Q

Give some causes of reactive eosinophilia

A

Parasitic infection
Allergic disease e.g. asthma, rheumatoid, polyarteritis, pulmonary eosinophilia
Underlying neoplasms esp. Hodgkin’s, T-cell NHL
Drugs (reaction erythema multiforme)

53
Q

What could be a primary cause of eosinophilia?

A

Chronic eosinophilic leukaemia

54
Q

What can cause monocytosis?

A

TB, brucella, typhoid
Viral: CMV, varicella zoster
Sarcoidosis
Chronic myelomonocytic leukaemia (MDS)

55
Q

What can cause lymphocytosis (raised lymphocytes)?

A

EBC, CMV, toxoplasma
Infectious hepatitis, rubella, herpes infections
Autoimmune disorders
Sarcoidosis

56
Q

What can cause lymphopenia?

A

Infection e.g. HIV
Auto immune disorders
Inherited immune deficiency syndromes
Drugs (chemotherapy)

57
Q

What is the cause if there are mature lymphocytes in the blood with no atypical cells?

A

Reactive/infectious cause

58
Q

What is the cause if there are mature lymphocytes in the blood in addition to small lymphocytes and smear cells?

A

CLL

NHL

59
Q

If there are lymphoblasts present in the peripheral blood what is the cause?

A

Acute lymphoblastic leukaemia

60
Q

Chronic lymphocytic leukaemia blood film

A

Mature lymphoid cells

61
Q

Acute lymphobastic leukaemia blood film

A

Immature lymphoid cells

62
Q

What is the light chain ratio/clonality in reactive lymphocytosis?

A

Polyclonal

Kappa & Lambda 60:40

63
Q

What is the light chain ratio/clonality in malignant lymphocytosis?

A

Kappa only or lambda only

99:1 malignant

Haematology (E) (14 decks)

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