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Medicine > Haematology > Flashcards

Haematology Flashcards

1
Q

Commonest cause of sideroblastic anaemia?

A

Primary acquired sideroblastic anaemia (myelodysplasia)

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2
Q

Congenital cause of sideroblastic anaemia?

A

X-linked defect in haem synthesis key enzyme (ALA-S mutation, delta-amino laevulinic acid synthase)

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3
Q

What is sideroblastic anaemia?

A

Ringed sideroblasts (erythroblasts with perinuclear Fe-engorged mitochondria). Inadequate marrow use of Fe for Haem synthesis despite enough/raised Fe. Cannot incorporate Fe into protoporphyrin.

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4
Q

Causes of acquired sideroblastic anaemias?

A

Myelodysplastic syndrome
Drugs: chloramphenicol, cycloserine, isoniazid, linezolid, pyrazinamide
Toxins: EtOH, Pb.
Pyridoxine or copper deficiency.

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5
Q

Management of acute iron poisoning?

A 
Gastric lavage (within 1-2h), ?whole bowel irrigation. 
IV desferrioxamine (chelates Fe and reduces risk of liver damage)
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6
Q

Features of Pb poisoning?

A

Clinically: abdo pain, constipation, anaemia, peripheral neuropathy, blue line of gums.
Bloods: punctate basophilia (undegraded RNA), haemolysis signs. Marrow ringed sideroblasts.

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7
Q

Management of sideroblastic anaemia?

A

Symptomatic. Regular transfusion and Fe chelation.
Inherited form: ?respond to pyridoxine (Vit B6), cofactor for ALA-S.
For myelodysplastic syndromes (esp with Ch5q deletion) -> lenalidomide.

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8
Q

Clinical manifestations of Sickle cell disease?

A

SICKLE Swellings and splenic sequestration
Infections and infarctions
Chronic haemolysis, Crises (vaso-occlusive, aplastic, cholelithiasis)
Kidneys
Lungs
Eyes, Erection

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9
Q

Causes of G6PD deficiency crises?

A

Acidosis (DKA)
Infections
Oxidant drugs (antimalarial, sulfonamides, synthetic Vit K, nitrofurans)
Fava beans

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10
Q

What are heinz bodies? How do you see them

A

Precipitate of oxidised, denatured Hb. Need an extra stain to see them (e.g. New methylene blue or bromocresol green)

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11
Q

Causes of heinz bodies?

A 
NADPH deficiency
G6PD deficiency crisis
Chronic liver disease
Alpha-thalassaemia
Hyposplenism/asplenia.
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12
Q

Investigations for haemolytic anaemia?

A

FBC + MCV
Reticulocyte count
Peripheral blood film
Urine dip for haemoglobinuria, haemosiderinuria
?LFTs conj v unconj bilirubin. Gallstones?

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13
Q

What are iron studies?

A

Ferritin
Serum Fe
Transferrin/TIBC
Transferrin saturation

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14
Q

What is RDW?

A

Red cell distribution width. Increased if anisocytosis (commonly due to nutritional deficiencies)

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15
Q

Hb 110, MCV 110, RDW raised. What is the likely diagnosis?

A

Macrocytic anaemia due to megaloblastic. E.g. B12/folate def.
Normal RDW = all other macrocytoses.

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16
Q

Hb 110, MCV 70, RDW raised. What is the likely diagnosis?

A

Fe deficient anaemia. Microcytic anaemia with RDW normal would indicate heterozygote thalassaemia (or HbE trait, or ACD)

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17
Q

Causes of raised RDW?

A

Folate and B12 def. Immune haemolytic anaemia, myelodysplastic syndromes, (liver disease)
Fe def. Sickle cell.
Dimorphic anaemia

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18
Q

How to distinguish ACD v Fe def anaemia?

A 
TIBC/transferrin: up in Fe def, low in ACD (don't want circ Fe)
Serum ferritin (=Fe stores): low in Fe def; up in ACD
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19
Q

How to differentiate sideroblastic anaemia from other microcytic anaemias?

A

High serum Fe, high transferrin sats %, high serum ferritin (stores). Low TIBC/transferrin.

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20
Q

How to distinguish thalassaemia from other microcytic anaemias?

A

Most of iron studies are normal (ferritin, transferrin, transferrin sats, serum Fe) RDW likely to be normal.
RBC count is raised.
Film: target cells and Howell-jolly bodies.

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21
Q

What happens to iron in acute inflammation?

A

Acute phase proteins (APP): ferritin and hepcidin (store Fe, decrease intake)
Negative APP: transferrin.
Decrease in circ Fe > decrease of transferrin.

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22
Q

What is Plumber Vincent Syndrome?

A

Oesophageal strictures seen in Fe def.

23
Q

What does hepcidin do?

A

Stops absorption of Fe from gut. (Via inhibition of ferroportin on BM of enterocytes) also inhibit Fe uptake by transferrin.

24
Q

How to diagnose haemochromatosis?

A

Liver biopsy showing evidence of heavy Fe deposition and hepatic fibrosis. OR genetic testing

25
Q

Complications of haemochromatosis?

A

Diabetes mellitus, cirrhosis, DCM, hypogonadism (?pituitiary), pseudogout, impotence,

26
Q

How to distinguish B12 from folate deficiency?

A

Urine methylmalonic acid increased in B12 and normal in folate.

27
Q

Should you correct B12 or folate alone?

A

You can do BUT DO NOT give folate before checking if the B12 is not. B12 BEFORE FOLATE. Or risk SCDC

28
Q

What is the cause and effect of increased plasma homocysteine?

A

B12 and/or folate def.

Increased vasc plaques, and thrombosis

29
Q

How to differentiate causes of B12 def?

A

Schilling test +/- corrected for IF.

Pernicious anaemia v intestinal disease.

30
Q

Pentad of TTP?

A 
Thrombocytopaenia
Renal failure
Fever
CNS def
MAHA
31
Q

Cause of TTP?

A

ADAMTS13-14 def.

32
Q

Triad of HUS

A

Renal failure MAHA, thrombocytopaenia

33
Q

Causes of thrombocytopaenia

A

Increased destruction: immune, non-immune

Decreased production

34
Q

Causes of decreased production of platelets:

A

Marrow failure
Aplastic anaemia
B12 def
Infx (parvovirus B19)

35
Q

Causes of women with ?haemophilia

A

severe vWD?
Parental haemophilia
X-inactivation

36
Q

Causes of thrombocytopaenia?

A

Increased destruction: immune (infx, auto, allo) ; non-immune
Decreased production: I AM BACK

37
Q

Immune causes of platelet destruction?

A

Infection: HIV, EBV, dengue fever
Allo: transfusion, heparin induced, HDN
Auto: primary ITP; secondary SLE, cancer, PNH

38
Q

Nonimmune causes of increased platelet destruction?

A

DIC

HUS and TTP

39
Q

Causes of decreased platelet production?

A 
Infections Parvovirus B19
Aplastic
Marrow failure
B12 def
Alcohol
Cirrhosis/liver failure
Kidney failure
40
Q

Acquired Coagulation disorders

A 
DIC
Liver failure
Warfarin
Vit K def
Autoantibodies: ITP, SLE, cancer, PNH?
41
Q

Hereditary coagulation disorders

A

Haemophilia or vWD

42
Q

Which factors in Vit K involved in action?

A

II, VII, IX, X

43
Q

All problems of blood transfusions

A 
GOT A BAD UNIT
Graft v Host
Overload (volume)
Thrombocytopaenia
Alloimmune 
BP unstable
Acute haemolytic transfusion reaction (AHTR)
Delayed haemolytic transfusion reaction (DHTR)
Urticaria
Neutrophilia
Infection
TRALI (transfusion related acute lung injury)
44
Q

Problems after transfusing 1 unit of blood?

A 
Don't Forget To Ask About Getting Itchy
Delayed haemolytic transfusion reaction
Febrile non-haemolytic transfusion reaction
TRALI
Acute haemolytic transfusion reaction
Allergic reaction
Graft v host disease
Infection: Hep B/C/E; HIV; malaria; CMV; syphilis; vCJD
45
Q

Problems after massive transfusion

A 
BACK CHaT
Bleeding
Acidosis (lysis)
Ca low (chelators)
K up (lysis)
Circulation overload
Haemochromatosis
All the immune stuff (AHTR/DHTR/TRALI/GvH/allergic)
Thermia (hypothermia)
46
Q

Causes of normocytic anaemia?

A 
<3% reticulocytes: BREAM
>3% reticulocytes: haemolytic anaemia
Blood loss (early)
Renal (RDW wide) due to low EPO 
Early Fe def anaemia/ACD
Aplastic anaemia
Malignancy
47
Q

Causes of haemolytic anaemia?

A 
Intrinsic to RBC
- inherited: Hb, metabolism, membrane
- acquired: PNH, infx
Extrinsic to RBC
- sequestration
- immune (allo, auto, drugs)
- mechanical (macro, micro)
48
Q

Causes of aplastic anaemia

A

Congenital: Fanconi’s anaemia, TAR, dyskeratosis congenita etc
Acquired:
- idiopathic
- secondary: post-chemo/radio; idiosyncratic; drug related (carbimazole); chemical (benzene); infections (EBV, parvovirus B19, viral hepatitis)
- asn with malignancy e.g. ALL, thymoma
- PNH

49
Q

Drugs that cause aplastic anaemia

A 
Carbamizole
Chloramphenicol
Sulphonamides
Chlorpromazine
Gold
50
Q

Causes of intrinsic inherited haemolytic anaemias?

A

Inherited

  • Hb: HbS, HbC
  • metabolism: G6PD, PKD
  • membrane (MCH up): spherocytosis or elliptocytosis
51
Q

Causes of intrinsic acquired haemolytic anaemias?

A

PNH: PIGA def
Infections: malaria, clostridia, viral, babesiosis

52
Q

Causes of mechanical haemolytic anaemia?

A 
Macro: 
- valves (AS or replacement/metallic); 
- March; 
- Burns/hyperthermia
Micro: DIC, TTP, HUS
53
Q

Causes of immune mediated haemolytic anaemia?

A

Drug related: penicillin, quinine, methyl-DOPA
Auto: warm (IgG); cold (IgM)
Allo: transfusion, haemolytic disease of newborn (Rh/ABO)

54
Q

Causes of microcytic anaemia?

A 
TAILS
Thalassaemia
Anaemia of chronic disease
Iron def
Lead poisoning
Sideroblastic

Medicine (16 decks)

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