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Haem - pathology > 2 > Flashcards

2 Flashcards

1
Q
  1. Q. What is the normal plasma volume of a RBC?
A

A. Hb Male Hb = 13.1 – 16.6 g/dL

B. Female Hb = 11.0 – 14.7 g/dL

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2
Q
  1. Q. What is anaemia? Describe the compensatory/pathological consequences?
A 
A.	Reduced haemoglobin = reduced O2 transport = tissue hypoxia 
B.	Compensatory changes:
a.	Increase tissue perfusion
b.	Increase O2 transfer to tissues
c.	Increase red cell production
C.	Pathological changes:
a.	Myocardial fatty change
b.	Fatty change in liver
c.	Aggravate angina/claudication
d.	Skin and nail atrophic changes
e.	CNS cell death (Cortex and basal ganglia)
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3
Q
  1. Q. What do reticulocytes measure?
A

A. Measures how fast RBC/reticulocytes are made by the bone marrow and released into the blood. After aprox. 2 days they develop into mature RBCs. When there is increased blood loss/removal of blood the retic count rises.

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4
Q
  1. Q. Name a cause for each of the following A) Microcytic anaemia B) Normocytic C) Macrocytic
A 
A.	Microcytic anaemia
a.	Iron deficiency
b.	Chronic disease
c.	Thalassaemia
B.	Normocytic anaemia
a.	Acute blood loss
b.	Anaemia of chronic disease
c.	Combined haematinic deficiency
C.	Macrocytic anaemia
a.	B12/folate deficiency
b.	Alcohol excess/liver disease
c.	Hypothyroid
d.	HAEMATOLOGICAL
i.	Antimetabolite therapy
ii.	Haemolysis
iii.	Bone marrow failure
iv.	Bone marrow infiltration
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5
Q
  1. Q. What are haemoglobinpathies?
A

A. disorder of quality (sickle cell), disorder of quantity (alpha or beta thalassaemia)

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6
Q

three types of haemoglobin?

A

A. Normal Hb: 2alpha, 2 beta chains
B. Foetal Hb: 2alpha, 2 gamma
C. Embryo: HbE gower-1 (2 zeta, 2 epsilon) and then HbE gower-2 (2 alpha, 2 epsilon), HbE Portland-1 (2 zeta, 2 gamma)

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7
Q
  1. Q. What is sickle cell disease? In what genetic state do they arise?
A

A. Homozygous state – recessive (most common inherited disease in England)
B. Amino acid change: thiamine for adenine in 6th codon of b globin gene (results in change of valine for glutamine)
C. Results in sickle-like-shaped RBCs

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8
Q
  1. Q. What complications are often seen in adults and children in sickle cell disease?
A

A. Common complications in children and adults: chronic haemolytic anemia, infections, painful crisis, splenic sequestration (trapped RBCs), acute chest syndrome, stroke (ischemic and hemorrhagic, aplastic crisie)
(Onset at 6 months/12 months due to HbF in infant haemoglobin which protects sickle harmoglobin.)
B. Common complications specific to adults: avascular necrosis, priapism, pulmonary arterial hypertension, sickle nephropathy, ocular disease, iron overload, leg ulcers

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9
Q
  1. Q. What disease are sickle cell carries protected against? In what areas is this disease most prevalent?
A

A. Falciparum malaria

B. South/west Africa, Caribbean

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10
Q
  1. Q. Describe the management of sickle cell disease
A

A. Acute: painful crisis: painkillers, sickle chest syndrome: vaso-occlusive crisis of pulmonary vasculature – CXR may show pulmonary infiltrate), stroke (higher risk in children 4-14 and other vasculature problems)
B. Chronic: renal impairment: blood vessels subject to chronic occlusion, pulmonary HTN, joint damage
C. Disease modifying treatment: transfusion, hydrocarbamide (switches on baby haemoglobin HbF), stem cell transplant (many associated comorbidities)

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11
Q
  1. Q. What is thalassaemia? Where is the highest prevalence worldwide?
A

A. Globin chain disorders resulting in diminished synthesis of one or more globin chains with consequent reduction in the haemoglobin
B. Mostly recessive, some reports of heterogeneous (dominant) – alpha thalassemia and beta thalassemia: severity depends on how many of the four genes for alpaha globin or beta globin are missing
C. Greece, Italy/Turkey, Eastern Europe, Middle east (India, Thailand)

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12
Q
  1. Q. Describe the clinical classifications of thalassaemia
A

A. Severity depends on how many of the four genes for alpaha globin or beta globin are missing. Classed by:
B. Thalassaemia Major
i. Transfusion dependent
C. Thalassaemia Intermedia
i. Less severe anaemia and can survive without regular blood transfusions
D. Thalassaemia Carrier/heterozygote
i. Asymptomatic

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13
Q
  1. Q. Describe the natural progression of beta-thalassaemia major over time
A

A. Presentation 6-12 months –> Inadequate Hb production –> Severe anaemia and hypoxia –> Lifelong blood transfusions –> Progressive increase in body iron load –> Hemosiderosis –> Iron deposits in liver and spleen –> Liver fibrosis and cirrhosis –> deposits in endocrine glands and heart –> Diabetes, HF –> Cardiac hemosiderosis –> Premature death

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14
Q
  1. Q. Describe the classic clinical presentation of beta-thalassaemia
A

A. Age: 6-12 months
B. Severe symptoms: failure to feed, listless, crying, pale
C. Bloods: HB 40-70 g/l, MCV & MCH very low
D. Blood film: large and small (irregular) very pale red cells, NRBC
E. Hb F > 90% (neonatal sample)

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15
Q
  1. Q. What is the management of thalassaemia major?
A

A. Regular transfusion
B. Iron chelation: transfusion can lead to iron overload (drugs used for removal of excess iron)
C. Endocrine supplementation (gonadal function/diabetes/growth and puberty/ Vit D/calcium, PTH, thyroid, dexa screening)
D. (fertility)
E. Bone health
F. Psychological support

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16
Q
  1. Q. How does alpha thalassaemia differ to beta thalasseaemia?
A

A. Alpha: variable, range of severity
B. Common carriage – eastern med and far east
C. Pale skin, weakness, fatigue, hepatosplenomegaly, jaundice

17
Q
  1. Q. What are membranopathies?
A

A. Deficiency of red cell membrane proteins caused by a variety of genetic leisons
B. Spherocytosis (vertical interactions) & elliptocytosis (horizontal interactions) most common
C. Autosomal dominant
D. Presentation: neonatal jaundice, mild to mod haemolytic anemia (exacerbated during infection), gallstones (linked to ilirubin/jaundice), folic acid and plenectomy in some cases

18
Q
  1. Q. What infection can be fatal in haemolytic anaemia?
A

A. Parvovirus: common infection in children, occurs in edpidemics
B. “Slapped cheek syndrome” – leads to decreased RBC production, dramatic Hb drop in pts who already have reduced RBC lifespan

19
Q
  1. Q. How may an enzymopathies affect a patient?
A

A. Enzymes: provide fuel for RBC, generates redox capacity to protect the cells
B. Inherited enzyme deficiencies lead to shortened RBC lifespan from oxidative damage (also have malarial protection)
C. G6PD deficiency (usually mild) and pyruvate kinase deficiency most common
D. Presentation: haemolysis, jaundice, anaemia
E. Precipitated by broad beans, infections, drugs
F. Symptomatic pts are rare – usually self-limiting
(Some important drug interactions: Primaquine, Sulphonamides, Nitrofurantoin, Quinolones, Dapsone G)

20
Q
  1. Q. Name some causes of polycythaemia
A 
A.	(Too many RBCs)
B.	Reactive/Secondary
a.	Smoking
b.	Lung disease
c.	Cyanotic heart disease
d.	Altitude
e.	Epo/Androgen excess
C.	Primary/Proliferative
a.	Polycythaemia Rubra Vera (myeloproliferative disorder: overactive bone marrow: RBCs, also WBCs and Plts –thrombosis & plethoric appearance (ruddy, swollen), itchy, splenomegaly, abnormal FBC – aspirin – venesection – bone marrow suppressive drugs
21
Q
  1. Q. Name some causes of neutrophillia
A

A. Reactive: Infection, Inflammation, Malignancy

B. Primary: CML

22
Q
  1. Q. Name some causes of thrombocytosis
A

A. Reactive: Infection, Inflammation, Malignancy

B. Primary: Essential Trombocythaemia

23
Q
  1. Q. Name a cause of severe neutropenia
A 
A.	Underproduction
a.	Marrow failure
b.	Marrow infiltration
c.	Marrow toxicity e.g. drugs
B.	Increased removal
a.	Autoimmune
b.	Felty’s syndrome
c.	Cyclical
24
Q
  1. Q. What are platelets? How do they form?
A

A. Fragments of megakaryocytes (mother of platelets), anucleate cells, aprox 4000 per MK cell – form in bone marrow
B. Life span: 7-10 days
C. Normal count 150-400 x 10^9/L
D. Phagocytosed by splenic macrophages
E. (produced by liver, removed by spleen)

25
Q
  1. Q. Name some surface glycoproteins of platelets
A

A. ABO, HPA, HLA Class I, glycoproteins (e.g. GP1a)

Haem - pathology (5 decks)

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