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MBBS II HIS > L32 - Practical approach to the investigation of haematological disorders > Flashcards

L32 - Practical approach to the investigation of haematological disorders Flashcards

1
Q

Classify and list common causes of hyper- and hypoproliferative anaemia?

A

Hyperproliferative:

  • Thalassaemias/haemoglobinopathies/membranopathy/ enzymopathy
  • Haemolytic anaemia / haemolysis

Hypoproliferative:

  • Nutrition deficiency
  • Primary BM failure
  • Drug/ toxin-induced
  • Primary BM pathologies (infiltrative)
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2
Q

List all the subtypes of Immune- cause haemolytic anaemia?

A

1) Autoimmune: e.g. SLE, CLL, Mycoplasma infection, lymphomas…etc
 Warm – primary vs. secondary
 Cold – primary vs. secondary (lymphoma, autoimmune diseases)
 Drug-induced (e.g. methyldopa)

2) Alloimmune (antibodies against transfused RBCs):
- Acute hemolytic transfusion reaction (e.g. ABO)
- Delayed HTR - anti-Jka /Jkb

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3
Q

List 6 causes of Microangiopathic Haemolytic anaemia?

A
    • Disseminated intravascular coagulation (DIC)
    • Thrombotic thrombocytopenic purpura (TTP)
  • Prosthetic heart valves
  • Malignant hypertension
  • Systemic vasculitis

-Pregnancy-related complications

(Haemolytic-uraemic syndrome (HUS))

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4
Q

List some causes of Thrombotic thrombocytopenic purpura (TTP)?

A

1) Immune: Idiopathic (def. ADAMTS13 = cannot cleave vWF) or secondary to autoimmunity

2) Secondary:
- Malignancy related (TMA)
- DRUGS: Ticlopidine, Cyclosporine A, Clopidogrel…etc
- HSCT rejection

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5
Q

What nutrient def. can cause hypoproliferative anaemia?

A
  1. Iron deficiency 2. Vitamin B12 / folate deficiency
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6
Q

Which primary BM pathologies can cause hypoproliferative anaemia by infiltration?

A
  1. MDS
  2. Leukaemia
  3. Lymphoma
  4. Myeloma

(myeloproliferative neoplasm (e.g. polycythemia vera): INCREASES hematopoiesis )

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7
Q

List 2 primary BM failures causing pancytopenia?

A
  1. Aplastic anaemia (autoimmune)

2. inherited bone marrow failure syndromes (IBMFS)

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8
Q

Which blood metric is critical to determine hyper vs hypoproliferative anaemia?

A

Peripheral blood film examination with estimation of reticulocyte count***

Low = can indicate BM failure/ nutrient def.

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9
Q

Which 4 serum molecules indicate haemolysis?

A
  • Increased total bilirubin
  • Increase LDH
  • Decrease Haptoglobin
  • Decrease Methemalbumin
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10
Q

Why is Haptoglobin and methemalbumin decreased due to haemolysis?

A
  • Haptoglobin take up Hb to form complex&raquo_space; cannot pass renal glomeruli&raquo_space; prevent excretion
  • Methemalbumin bind to Hb after Haptoglobin is used up
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11
Q

List 3 lab tests to further ddx haemolysis cause?

A

 Blood film/PBS (red cell morphology: e.g. spherocytosis fragmentation, signs of oxidative hemolysis)
 Direct, indirect antiglobulin tests
 G6PD assay

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12
Q

List the most common causes of warm and cold immune haemolysis?

A

 Cold: infections

 Warm: autoimmune disease, drugs

Both: idiopathic or part of LPD

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13
Q

Give 4 principle causes of bleeding tendencies?

A

 Clotting factor deficiency– coagulopathy, hemophilia A/B

 Platelet – quantitative / qualitative functional disorders

 Drugs: functional disorder: warfarin, factor inhibitors…

 Connective tissue / collagen vascular disease

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14
Q

What tests should be done to ddx platelet or coagulation disorders?

A

 PBS/Blood film

 Clotting profile: PT, APTT, TT

 Other ancillary investigations (e.g. VWF ristocetin cofactor assay)

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15
Q

List some causes of isolated thrombocytopenia?

A

1) Primary BM failure (rare to just involve one lineage, except congenital amegakaryocytic thrombocytopenia)

2) Peripheral causes:
- Immune mediated destruction: Autoimmune Thrombocytic Purpura + Alloimmune Thrombcytopenia
- Non-immune: increased sequestration or consumption (DIC, TMA, TTP)
- Drug induced

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16
Q

List some causes of thrombocytopenia asso with other cytopenias?

A

Peripheral:
- Hypersplenism (sequestration and consumption)
- Autoimmunity against HSC (e.g Aplastic anemia)
Primary:
- BM pathology (infiltration, e.g. leukemia, lymphoma)
- Primary BM failure

17
Q

List clonal or reactive causes of high WBC count in peripheral blood?

A

Clonal:

  • Blasts >=20%: acute myeloid leukemia, acute lymphoblastic leukemia
  • Blasts <20%: other myeloid malignancies: MPN, MDS

Reactive:

  • Infection
  • Inflammatory/autoimmune
  • Paraneoplastic/ solid tumour reaction
18
Q

Which types of acute leukemia doesnt present with Blasts >=20%?

A
  1. Acute promyelocytic leukemia
  2. Acute myeloid leukemia with t(8;21) translocation
  3. Acute myeloid leukemia with t(16;16) translocation
19
Q

Characteristic genetic mutation of APL?

A

t(15;17) PML-RARA

20
Q

Describe the morphology of WBC in APL?

A

Abnormal promyelocyte (Faggot cells: bilobed nuclei, Auer rods, abundant malignancy features)

– no need to see >20% blasts to dx

21
Q

Treatment regimen for APL?

A

ATRA/ Arsenic trioxide immediately +/- supportive transfusion, esp. platelet, plasma

+ Correct coagulopathy, thrombocytopenia (DIC, restore platelet)
+ Treat any underlying infection
+ Treat and prevent tumor lysis syndrome (TLS) e.g. xanthine oxidase

22
Q

Which procedures must not be done for APL?

A

Do not perform invasive procedures

Do not give granulocyte colony stimulating factor (G-CSF)

Do not stop ATRA/ATO

23
Q

Which lab tests for Dx of APL?

A

 Bone marrow examination with cytogenetics

 Molecular genetics (e.g. RT-PCR for PML-RaRα)

 PBS: Faggot cells and Auer rods

24
Q

List some ADR of ATRA/ATO for APL treatment?

A

General:
 Skin rash  Headache  Nausea, vomiting

ATO specific:
 Hepatitis
 Prolonged QTc = arrhythmia
 Risk of herpes zoster reactivation

25
Q

MCV of Inherited sideroblastic anaemia?

A

Microcytic

occasionally acquired due to nutritional deficiency

26
Q

Define the stain used to ID sideroblastic anaemia, histological and marrow appearance?

A
  1. Iron stain >1/3 of erythrocyte: Dysplastic with cytoplasmic vacuoles in erythroid and myeloid precursors
  2. Hypercellular marrow with Nucleus-cytoplasmic asynchrony
27
Q

List 2 inherited membranopathies that cause haemolytic anaemia?

A

Her. elliptocytosis

Her. Spherocytosis

28
Q

Describe the absorption of copper and its function?

A

Dietary copper absorbed at small bowel

Transport in plasma by albumin/ Transcuperin to liver

Biliary excretion or carried by Ceruloplasmin to tissue

> co-factor for metabolism, incl: erythropoiesis and heme synthesis

29
Q

List 1 acquried and 4 reversible causes of sideroblastic anaemia?

A

Acquired = MDS

Reversible:
Alcoholism 
Drugs (e.g. isoniazid)
**Copper deficiency**
**Pyridoxine def.**
30
Q

3 mechanisms of bleeding in APL?

A
  • Marrow infiltration&raquo_space; reduced platelet production
  • Increased consumption of plt due to DIC
  • Hyperfibrinolysis due to increased Annexin II&raquo_space; deplete fibrinogen
31
Q

List some plasma markers of hypercoagulation?

A

Increased:

  • Prothrombin
  • D-dimer
  • Thrombin- Antithrombin complexes (TAT)
  • Fibrinopeptide A (FPA)
32
Q

List some plasma markers of fibrinolysis ?

A
  • Low plasminogen, a2-antiplasmin, fibrinogen
  • ## Increased D-dimer, Fibrin degradation products (FDP)
33
Q

APL usually presents with leucocytosis. T or F?

A

False

Only 10% APL = high WBC at presentation
Usually leukopenia

MBBS II HIS (30 decks)

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