3) Immunodeficiency and Bone Marrow Failure Flashcards

1
Q

Give examples of causes of acquired immunodeficiencies:

A
Chronic lymphatic leukaemia
Myeloma
HIV
Chemotherapy 
Radiotherapy
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2
Q

Which acquired immunodeficiencies affect B cells?

A

CLL
Lymphoproliferative disorders
Myeloma
Nephrotic syndrome

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3
Q

Which acquired immunodeficiencies affect T cells?

A

HIV
Chemo
Immunosuppression e.g. Transplantation
Hodgkin’s disease

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4
Q

Which acquired immunodeficiencies affect both B and T cells?

A

Radiotherapy
CLL
Malnutrition

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5
Q

Which acquired immunodeficiencies affect neutrophils?

A

Neutropenia

Myelodysplasia

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6
Q

What fungal infections can occur in HIV disease?

A

Pneumocystis jirovecii
Candida albicans
Aspergillosis fumigated
Cryptococcus neoformans

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7
Q

What mycobacterial infections can occur in HIV disease?

A

Tuberculosis

Mycobacterium avium-intracellulare

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8
Q

What parasitic infections can occur in HIV disease?

A

Cryptosporidia

Toxoplasma gondii

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9
Q

What viral infections can occur in HIV disease?

A

Cytomegalovirus
Herpes zoster
Herpes simplex

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10
Q

How can pneumocystis pneumonia be diagnosed?

A

Direct microscopy of broncho-alveolar lovage

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11
Q

How is pneumocystis pneumonia treated?

A

Cotrimoxazole

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12
Q

How is candidiasis diagnosed and treated?

A

Clinically by visualisation of white plaques

Fluconazole

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13
Q

What infections can cytomegalovirus cause and how is it treated?

A

Pneumonitis, oesophagitis, colitis, hepatitis

Acyclovir/gancyclovir

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14
Q

What are the sites of haemopoiesis in the fetus? (3 stages)

A

0-2 months - yolk sac
2-5 months - liver and spleen
5-9 months - bone marrow

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15
Q

What are the sites of haemopoiesis in infants?

A

Bone marrow in most bones

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16
Q

What are the sites of haemopoiesis in adults?

A

Bone marrow: vertebrae, ribs, sternum, sacrum, pelvis, proximal femur

17
Q

What is the difference between red and yellow marrow?

A

Red - contains haematopoietic tissue

Yellow - mainly fatty tissue

18
Q

How does the composition of marrow change as we age?

A

All marrow at birth is red

Half of marrow is red in adults, rest converted to yellow

19
Q

How can bone marrow be examined?

A

Trephine biopsy

Bone marrow aspiration

20
Q

Describe the development of a myeloblast into a neutrophil:

A

Myeloblast -> promyelocyte -> myelocyte -> metamyelocyte -> band cell -> neutrophil

21
Q

Describe the development of a proerythroblast into a red cell:

A

Proerythroblast -> early normoblast -> intermediate normoblast -> late normoblast -> reticulocyte -> RBC

22
Q

What is the red pulp of the spleen?

A

Sinuses lined by endothelial macrophage and cords

23
Q

What is the white pulp of the spleen?

A

Similar structure to lymphoid follicles

24
Q

What are the functions of the spleen?

A

Sequestration and phagocytosis
Blood pooling
Extramedullary haemopoiesis
Immunological function

25
Q

What are the causes of a massive splenomegaly?

A

Chronic myeloid leukaemia
Myelofibrosis
Chronic malaria
Schistosomiasis

26
Q

What are the causes of a moderate splenomegaly?

A

Lymphoproliferative disorders
Myeloproliferative disease
Portal hypertension
Glandular fever

27
Q

What are the causes of a mild splenomegaly?

A

Infectious hepatitis
Endocarditis
Sarcoidosis
Autoimmune diseases - SLE, ITP

28
Q

What can cause hyposplenism?

What will be seen on blood film?

A

Splenectomy
Sickle cell disease
Coeliac disease

Can see Howell Jolly bodies

29
Q

What are some causes of thrombocytopenia?

A

Reduced production - B12/folate def, bone marrow cancer, aplastic anemia, chemo, HIV

Increased removal or use - immune destruction (ITP), DIC, TTP, splenic pooling

30
Q

What is immune thrombocytopenic purpura and how is it treated?

A

Autoantibodies against glycoproteins on platelets

Treated with immunosuppression

31
Q

What are the consequences of severe thrombocytopenia?

A
Easy bruising
Purpura 
Mucosal bleeding 
Severe bleeding after trauma or procedures
Intracranial haemorrhage
32
Q

What are the causes of neutropenia?

A

Reduced production: B12/folate def, cancer of bone marrow, aplastic anemia, radiation, drugs, viruses, congenital

Immune destruction, sepsis, splenic pooling

33
Q

What are the consequences of neutropenia?

A

Severe life threatening bacterial and fungal infections

Mucosal ulceration

34
Q

What are the causes of pancytopenia?

A

Reduced production: B12/folate def, cancer of bone marrow, aplastic anemia, radiation, drugs, viruses, congenital

Immune destruction, splenic pooling

35
Q

What is aplastic anaemia?

A

Pancytopenia with a hypocellular bone marrow in the absence of an abnormal infiltrate and with no fibrosis

36
Q

What malignancies can cause pancytopenia?

A
Acute and chronic leukaemia
Lymphoma
Myeloma
Myelofibrosis
Prostate, breast and lung cancer
37
Q

What are some symptoms of pancytopenia?

A

Anaemic - fatigue, dizziness, shortness of breath
Bleeding and bruising
Infection, ulcers, fevers