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Haematological Changes in Systemic Disease Flashcards

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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
Give an example of a secondary disorder that leads to FVIII deficiency
anti-FVIII auto-antibodies in acquired haemophilia A --> FVIII deficiency
26
Fe deficiency is
Bleeding until proven otherwise
27
If Fe is found and there is an underlying malignancy, what will the blood tests show?
Fe deficiency Microcytoic hypochronic anaemia Reduced ferritin, transferrin saturation Raised total iron binding capacity (TIBC)
28
What may cause occult blood loss?
GI cancers - gastric, colorectal | Urinary tract cancers - renal cell carcinoma, bladder cancer
29
What is a leuco-erythroblastic anaemia?
Variable degree of anaemia
30
What are the blood film features in leuco-erythroblastic anaemia?
Teardrop RBCs (+aniso and poikilocytosis) Nucleated RBCs Immature myeloid cells
31
Normal peripheral blood film features
No nucleated RBCs | Mature cells
32
Leucoerythroblastic film
Tear drop poikilocytes Nucleated RBCs Myelocyte
33
What must have happened for a leucoerythroblastic film?
Bone marrow infiltration
34
What are the causes of bone marrow infiltration?
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
What are the common laboratory features of all haemolytic anaemias?
``` Anaemia (though may be compensated) Reticulocytosis Unconjugated bilirubin raised (pre-hapatic) LDH raised Haptoglobins reduced ```
36
What are the groups of haemolytic anaemias?
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
Give examples of primary haemolytic anaemias
Membrane e.g. Hereditary Spherocytosis Cytoplasm/enzymes e.g. G6PD deficiency Haemoglobin e.g. Sickle cell disease (structural) Thalassaemia (quantitative)
38
What are the types of acquired (secondary) haemolytic anaemias?
``` Non immune (DAT -ve) Immune mediated (DAT +ve, direct antiglobulin test i.e. Coombs test) ```
39
What are the key features of immune haemolytic anaemia?
Spherocytes | DAT +ve (Coombs test)
40
What systemic diseases are associated with immune haemolytic anaemia?
Malignancy e.g. Lymphoma, CLL Auto immune e.g. SLE Infection e.g. mycoplasma Idiopathic
41
What are some non-immune causes of acquired haemolytic anaemia?
Infection e.g. malaria | Micro-angiopathic Haemolytic anaeia (MAHA) - underlying adenocarcinoma, haemolytic uraemic syndrome
42
Micro-angiopathic haemolytic anaemia blood film
RBC fragments | Thrombocytopenia
43
Why does MAHA cause RBC fragments?
Platelet activation --> fibrin deposition and degradation --> red cell fragmentation (microangiopathy) --> bleeding (low platelets and coag factor deficiency)
44
What types of white blood cells are found in bone marrow?
Blasts (myeloid & lymphoid) Promyelocytes Myelocytes
45
What white blood cells are found in peripheral blood?
Phagocytes: granulocytes - neutrophils, eosinophil, basophils. monocytes Immunocytes: T lymphocytes, B lymphocytes
46
What are the immediate concerns if immature cells are seen in the peripheral blood?
Leukaemia or metastatic cancer invading bone marrow
47
What can cause neutrophilia (raised neutrophil count)?
``` Pyogenic infection Corticosteroids Underlying neoplasia Tissue inflammation e.g. colitis, pancreatitis Myeloproliferative/leukaemic disorders ```
48
How would you identify a reactive/infection neutrophilia?
Neutrophilia + toxic granulation no immature cells
49
How would you identify a malignant neutrophilia?
Neutrophilia plus basophilia & immature cells myelocytes. Suggest a myeloproliferative (CML) Neutrophenia plus myeloblasts suggests acute leukaemia (AML)
50
Reactive neutrophilia blood film
Only mature cells, toxic granules in neutrophils
51
CML blood film
Neutrophilia Immature cells (myelocytes) Basophils
52
Give some causes of reactive eosinophilia
Parasitic infection Allergic disease e.g. asthma, rheumatoid, polyarteritis, pulmonary eosinophilia Underlying neoplasms esp. Hodgkin's, T-cell NHL Drugs (reaction erythema multiforme)
53
What could be a primary cause of eosinophilia?
Chronic eosinophilic leukaemia
54
What can cause monocytosis?
TB, brucella, typhoid Viral: CMV, varicella zoster Sarcoidosis Chronic myelomonocytic leukaemia (MDS)
55
What can cause lymphocytosis (raised lymphocytes)?
EBC, CMV, toxoplasma Infectious hepatitis, rubella, herpes infections Autoimmune disorders Sarcoidosis
56
What can cause lymphopenia?
Infection e.g. HIV Auto immune disorders Inherited immune deficiency syndromes Drugs (chemotherapy)
57
What is the cause if there are mature lymphocytes in the blood with no atypical cells?
Reactive/infectious cause
58
What is the cause if there are mature lymphocytes in the blood in addition to small lymphocytes and smear cells?
CLL | NHL
59
If there are lymphoblasts present in the peripheral blood what is the cause?
Acute lymphoblastic leukaemia
60
Chronic lymphocytic leukaemia blood film
Mature lymphoid cells
61
Acute lymphobastic leukaemia blood film
Immature lymphoid cells
62
What is the light chain ratio/clonality in reactive lymphocytosis?
Polyclonal | Kappa & Lambda 60:40
63
What is the light chain ratio/clonality in malignant lymphocytosis?
Kappa only or lambda only | 99:1 malignant

Haematology (E) (14 decks)

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