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FRACP Clinical Exam > Haematology theory > Flashcards

Haematology theory Flashcards

1
Q

Thrombotic thrombocytopenic purpura (TTP):
[ ? ] Clinical: FAT RN (pentad)

Ix: ADAMTS13 deficiency, less cleaving vWF = microthombi, end organ ischemia.

Mx: PLEX, high dose steroid.

A

Fever
Haemolytic Anaemia
Thrombocytopenia
Renal failure
Neurological abnormalities

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2
Q

Drugs causing warm ab immunohemolytic anaemia (WAIHA)

A

Methyldopa
Pencillin
Quinidine
Fludarabine (CLL, NHL) unless combined with rituximab (CD20)

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3
Q

Drugs to avoid in G6PD

G6PD: oxidative hemolysis -> heinz bodies / bite cells, X-chromosomes (males), fava beans.

A

Antimalarials
Sulfonamides
Co-trimoxazozle
Dapsone

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4
Q

Risk factors for hemolytic anaemias

1.FHx + ethnicity
- greek or italian
- china/south east asian
- african/mediterranean/asian
2.Warm antibody hemolytic anaemia (2)
3.Tertiary syphilis
4.Secondary:
-DIC
-HUS
4.Trauma:
-Mechanical heart valve
-Joggers
5. Organ dysfunction:
-splenomegaly
-cirrhosis

A

FHx + ethnicity
- greek or italian: beta-thalassemia trait
- china/south east asian: alpha-thal
- african/mediterranean/asian G6PD

Warm antibody hemolytic anaemia
- SLE and other CTD
- Lymphoma (warm and cold ab)

Tertiary syphilis (paroxysmal cold hemoglobinuria)

Secondary:
-DIC: malignancy, renal graft rejection, HTN
-HUS (gastroenteritis, e.coli 0157:H7 infection not for abx).

Trauma:
Mechanical heart valve (aortic > mitral) with paravalvular leak
Joggers (external trauma)

Organ dysfunction:
-splenomegaly
-cirrhosis (spurr cell anaemia)

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5
Q

Fever in hemolytic anaemia (4)

A

Septicemia
Malaria-associated hemolysis
Acute sick cell crisis
TTP

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6
Q

Hemolytic anaemia blood film (3)

Coombs test:
-warm ab
-cold ab

A

Blood film:
- schistocytes (valve hemolysis, DIC, TTP or HUS)
- polychromasia
- hypochromic and microcytic (thalassemia).

Coombs test (immunohemolytic):
-warm: lymphoma (NHL), CLL, solid tumours (lung, colon, kidney, ovary), SLE and drugs, idiopathic
-cold: EBV, mycoplasma, hepatitis C, lymphoma, idiopathic
-paroxysmal cold hemoglobinuria (rare)

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7
Q

Multiple myeloma
1. Investigation
-Myeloma specific (4)
-CRAB
-MRI
-Prognostication (2)
2. Treatment
-Prevent renal failure
-Bone marrow suppression
-Bone lesions
-Systemic tx
. if eligible for SCT
. if not eligible for SCT
-SCT
3. Complications

A
  1. INVESTIGATION.
    -Myeloma specific:
    . BM bx (clonal plasma cells >10% or plasmacytoma)
    . SPEP (monoclonal globulin peak)
    . Serum/urine paraprotein (IgG > IgA > IgM)
    . Serum/urine light chains (bence-jones proteinuria and kappa:lambda ratio)
    -CRAB:
    . Calcemia (bone pain, abdo pain, dehydration, infection)
    . Renal disease: renal function and urate level
    . Anaemia: FBC and ESR
    . Bone lytic lesions: XR- skull, proximal long bones (also for fracture, osteoporosis)
    -MRI ?spinal cord compression or nerve root.
    -Prognostication:
    . Serum B2 microglobulin
    . Albumin
  2. TREATMENT
    -Indications
    . >60% plasma cells
    .serum free light chains >100
    . MRI lesion >5mm
    . hypercalcemia
    . renal failure
    . anaemia
    . lytic lesions

-Prevent renal faliure:
. Hydration, bicarbonate
. Allopurinol for urate nephropathy
. Caution IV contrast
-Bone marrow suppression:
. Avoid live vaccines
. EPO for anaemia
-Bone lesions:
. Bisphosphonates (Zolendronic acid)
. RTX (local irradiation, symptomatic benefit)
-Systemic tx if symptomatic or end-organ damage:
. IF eligible for SCT = steroid +/- thalidomide or CYC OR bortezomib + dex + CYC
. IF not eligible for SCT = melphalan + prednisone + (thalidomide/bortezomib/lenalidomide)
. SCT
3. COMPLICATIONS
-peripheral neuropathy (bort/ thali)
-thrombosis (lenali)
-infection
. secondary hypogammaglobulinemia (IVIG)
-bone
. Lesions
. Bisphosphonate complications
-steroid
-psychogical
-“geriatric” (frail)

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8
Q

DDx multiple myeloma (4)

A

MGUS
-serum paraprotein <30g/L
-bone marrow plasma cell infiltrate <10%

Smouldering multiple myeloma
-serum paraprotein >30g/L
-bine marrow plasmacytosis >10%
-absence of crab features

Waldenstroms macroglobulinemia
-monoclonal IgM peak EPG
-hyperviscosity
-similar symptoms
-Tx: plasmapharesis, prednisone, fludarabin

POEMS syndrome: osteosclerotic myeloma
-polyneuropathy
-organomegaly
-endocrinopathy
-monoclonal gammopathy
-skin changes

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9
Q

Complications autologous SCT: high dose chemo
D5-10: pancytopenic
D14: neutrophil

A

Organ toxicity (renal, VTE)
Infection:
-peritransplant (febrile neutropenia)
-late: HSV/VZV, PJP, CMV
-prevention
. Immunoglobulin
. Prophylaxis
. Vaccination
. Minimise immunosuppression (steroids)

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10
Q

Allogeneic transplant
Complications
-GVHD prophylaxis
-Pancytopenia
-VTE
-Mucositis
-CMV/EBV

A

Complications-
-GVHD prophylaxis (cyclosporin)
. Acute: skin, liver, GIT. Tx: high dose steroid, calcineurin inhibitor, MYC, ruxolitinib (JAKi). C/o- fungal, CMV, opportunistic.
. Chronic: D100+ scleroderma, as above + functional hyposplenism
-Pancytopenic
-VTE
-Mucositis (TPN/PCA)
-CMV / EBV reactivation: monitor viral load, tx: wean immunosupp, gan/valganciclovir (myelosupp), foscarnet.

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11
Q

CHA2DS2VASc
>1 mod
>2 high

A

CHF
HTN
Age >75
Diabetes
Stroke/TIA/VTE
Vascular disease
Age >65
Sex category (FEMALE)

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12
Q

HASBLED
>3 caution

A

HTN
Age >65
Stroke
Bleeding tendency or predisposition
Labile INR
Elevated renal / LFT
Drugs (blood thinners) or alcohol

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13
Q

Immune thrombocytopenia (ITP).
-autoimmune, anti-platelet IgG antibody
-clinical features
-investigation
-treatment

A

CLINICAL FEATURES.
-thrombocytopenia
-purpura
-haemorrhage

INVESTIGATIONS.
-platelet
-blood film
-bone marrow
-anti-platelet antibodies
-SLE (ANA, anti-dsDNA, complement)

TREATMENT.
-spontaneous improvement
-transfusion if acute bleeding + steroids
-if severe, IVIG
-splenectomy

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14
Q

Thrombocytopenias
A. Increased platelet destruction

B. Decreased platelet production

A

A. Increased platelet destruction.
-Immune thrombocytopenia
. idiopathic thrombocytopenic purpura
. secondary autoimmune thrombocytopenia
. drug-induced immune thrombocytopenia
. post-transfusion purpura
. viral infection (HIV)

-Consumptive thrombocytopenia
. TTP
. DIC
. HUS
-Hypersplenism (sequestration).

B. Decreased platelet production.
-bone marrow suppression (drugs, alcohol, toxins, infections)
-aplastic anaemia (pancytopenia)
-leukemias and bone marrow cancers
-megaloblastic anaemia (b12 defcieicnY)

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15
Q

Antiphospholipid syndrome

A

Abs (12 weeks):
1.anticardiolipin ab
2.lupus anticoagulant (prolong APTT)
3.anti-b2 glycoprotein

Features:
-thrombosis
-recurrent pregnancy loss
-haem: thrombocytopenia, haemolytic anaemia
-renal: acute thrombotic microangiopathy, chronic vaso-occlusive lesions
-cardiac: valve vegetation or thickening
-derm: livedo reticularis or racemosa, livedoid vasculopathy (recurrent painful skin ulcers)
-neuro: cognitive, subcortical white matter changes

Associated with SLE / Sjogrens.

Mx: LDA + LMWH in pregnancy, anticoagulation lifelong if VTE / infarct.

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16
Q

ITP v TTP v DIC

A

ITP - antiplatelet ab, steroid, IVIG, splenectomy

TTP- adamts13 deficiency, endothelial defect, plex (remove autoantibodies and large VWF), steroids

DIC- thrombin excess, increased d-dimer, increased INR, supportive.

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17
Q

Disseminated intravascular coagulation (DIC)

A

Pathophysiology:
tissue factor from vascular endothelial damage (trauma, chemo, bacteria, cytokine exposure)
activates factor VII -> extrinsic pathway coagulation cascade
thrombin and fibrin formed
clotting factors consumed -> excess bleeding
platelets trapped and consumed.

clinical history: severe infection, trauma, hepatic failure, obstetric complications, maligjnancy
VTE
bleeding
renal failure
end-organ damage (lungs, pulmonary haemorrhage or embolism)
coronary artery disease
stroke

Mx: secondary cause, mx active bleeding with platelet/ FFP, if not actively bleeding VTEp with heparin / LMWH.

18
Q

Microcytic anaemia

A

Thalassaemia

Iron deficiency: central pallor > 1/3

Anaemia of chronic disease
-hepcidin blocks ferroportin gate that lets iron into gut / macrophage.

Lead toxicity: basophilic stippling (polka dot)

Sideroblastic anaemia: ring sideroblasts.
1. Hereditary. Mx pyridoxine, blood tf for anaemia.
2. Acquired (drugs, chemo, MDS/MPN). Mx avoid agent, c/o diabetes.

19
Q

Macrocytic anaemia

A

EtOH
Liver disease
Myelodisplastic syndrome (sideroblastic)
B12/folate def
Drugs (phenytoin, cytotoxics, anti-virals, trimethoprim)
Reticulocytosis (haemolytic anaemia, bleeding)
Myeloma
Haemochromatosis
Smoking

Others- aplastic anaemia, COPD, anorexia, hypothyroidism, familial, pregnancy.

20
Q

Normocytic anaemia

A

Decreased production, less reticulocytes
-inflammation
-nutrient def
-EPO def
-bone marrow infiltration
-MDS
-chemo

Increased loss or destruction, more reticulocytes.
-acute bleed
-haemolysis

21
Q

Autoimmune haemolytic anaemia

A

Anaemia
Reticulocytes
Bilirubin (unconjugated)
Low haptoglobin
Direct antiglobulin test (DAT)

22
Q

Hereditary spherocytosis

A

Auto dominant
Red cell membrane protein abnormality
Splenic destruction
Pigment gallstones
Anaemia

Mx: splenectomy

23
Q

Splenectomy
-blood film
-acquired hyposplenism
. infarction
. atrophy/hypofunction
. infiltration

A

Blood film:
Howell Jolly bodies
Target cells
Spherocytes
Odd cells

Acquired hyposplenism:
Infarction: sickle cell, ET, PRV
Atrophy/hypofunction: coeliac, dermatitis herpetiformis, IBD, autoimmune (RA, SLE, GN, PBC, Sjogrens, MCTD, thyroiditis), irradiation, BMT/GVHD, HIV/AIDS
Infiltration:
Amyloid
Sarcoid
Leukaemia
Myeloproliferation

24
Q

Cold agglutinin disease (EBV, mycoplasma, lymphoma)

A

Cold agglutinin + haemolytic anaemia

  1. primary = distinct lymphoma
  2. secondary = infection, lymphoma
25
Q

Haemolytic uraemic syndrome

A

E.coli producing shiga-toxin
Strep pneumoniae
Atypical HUS

P- bloody diarrhoea, severe crampy abdo pain, nausea, vomiting.

VWF secreted from toxin-stimulated kidney endothelium

Thrombotic microangiopathy

26
Q

VWF deficiency
-genetic
-acquired

A

Acquired
-aortic stenosis
-LVAD
-ET (high plt)
-immune mediated
-malignancy
-hypothyroid

27
Q

Heyde syndrome (3)

A
  1. aortic stenosis
  2. acquired von willebran syndrome
  3. GI bleeding
28
Q

Heparin induced thrombocytopenia (HIT) 4T score

A

Thrombocytopenia
Timing of platelet count fall (d5-10)
Thrombosis or other sequelae (skin lesion, systemic reaction)
Other cause for thrombocytopenia (none)

PF4-heparin antibodies

29
Q

Haemophilia A and B

A

X-chromosome (males)

Replacement
Prophylaxis 2-3x week

Haem A : F VIII (inhibitors developed, emicizumab bypass, immunosuppression = CYC, RTX, IVIG)

Haem B: F XI

30
Q

Clotting cascade

Extrinsic pathway (PT) little

Intrinsic pathway (APTT) TENET

A

Vit K antagonist (warfarin) = factor 2, 7, 9, 10

Factor Xa inhibitors = rixaroxaban, apixaban, fondaparinux, LMWH (PT, modified anti-Xa assay)

Antithrombin = LMWH, UFH, dabigatran, bivalrudin (thrombin time, APTT)

31
Q

Warfarin + bleeding

Other anticoagulation and surgery (planned / unplanned)

A

Vit K
Prothrombinex
FFP

32
Q

Myeloproliferative neoplasm investigations

A

FBE: count and film

PCR/FISH: BCR-ABL1 for CML

PCR: JAK2, CALR, MPL

BM bx: hypercellular, fibrosis on reticulin stain

33
Q

CLL indications for tx

A

anaemia or immune thrombocytopenia
painful lymphadenopathy
B-symptoms: fever, chill, weight loss
lymphocyte doubling time <6/12
rapidly enlarging lymph nodes or organs
autoimmune hemolytic anaemia or idiopathic thrombocytopenic purpura refractory to immunosupp

34
Q

Acute myeloid leukaemia

A

Auer rods
Electrolytes, renal, liver, uric acid, LDH
Coagulation profiles
BM bx >20% blasts
Cytogenetics

35
Q

Myelodysplastic syndrome

A

Allogeneic HSCT
Transfusion
-iron chelation
-plt
GCSF, EPO
Chemo - azacitadine
Lenalidomine (chr 5q31 deletion)

36
Q

Acute intermittent porphyria

A

Auto dominant
Abnormal haem production
P: Abdo pain, neuropsych sx, peripheral neuropathy
R: triggered by alcohol, fasting, medication
D: Porphyrobilinogen, porphyrin in urine (days after attack).
M: IV haem c/o VTE risk. orthotopic liver transplant.
C: hepatic iron overload, liver fibrosis.

37
Q

Anticoagulation
-Factor Xa inhibitors
-Direct thrombin inhibitor

Debate against warfarin.

A

Factor Xa inhibitors: rivaroxaban, apixaban
Direct thrombin inhibitor: dabigatran

DEBATE AGAINST WARFARIN:
Novel anticoagulants advantages: rapid onset of action, fewer interactions with meds/food, no need for repeat bloods.
Disadvantages: not safe in renal failure,

38
Q

Haematology fundoscopy (2).

A

Choroid infiltration (chronic leukemia) or leopard skin.

Retinal gross venous dilatation and segmentation in hyperviscosity.

39
Q

Hodgkin’s v non-Hodgkin’s lymphoma (HL v NHL)

B symptoms poorer prognosis.

A

Reed-sternberg cells in Hodgkin’s, binucleate cells on light microscopy of biopsy.

B-symptoms: weight loss (>10% 6 months), fever, night sweats.

40
Q

Chronic lymphocytic leukemia (CLL)
-monoclonal proliferation (lymphocytes, B-cells).
-routine bloods with raised wcc
-bone marrow failure or constitutional symptoms
-Ix
-Binet system staging
-Mx

A

Ix:
-CT
-LN bx
-BM bx
-cytogenetics

Binet staging
-stage A <3 groups (watch and wait)
-stage B >3 groups
-stage C anaemia or thrombocytopenia

Mx:
-chemo: fludarabine and CYC
-monoclonal antibodies: rituximab
-Jaki: ibrutinib, idelalisib
-BM transplant

Complications:
-bone marrow failure
-autoimmune haemolytic anaemia
-recurrent chest infections
-acute transformation (richter’s syndrome) into aggressive lymphoma (DLBCL = fever, rapid node enlargement, severe LDH rise and high-grade NHL).

41
Q

Myeloproliferative disorders
-RBC
-WBC
-Plt
-Fibroblasts

CML dx: bloods, cytogenetic tests (Chr 9- philadelphia chromosome, BCR-ABL1 gene targetted by tyrosine kinase inhibitors e.g. imatinib).

A

RBC: PRV

WBC: CML

Plt: Essential thrombocythemia

Fibroblasts: myelofibrosis

42
Q

Purpuric / petechial rash

A

Febrile
1. palpable (infection / HSP)
-meningococcal
-disseminated gonococcal
-endocarditis
-henoch-schonlein purpura (IgA vasculitis, arthralgia large joints, nephritis, HTN).

  1. non-palpable
    -purpura fulminans
    -DIC
    -TTP

Afebrile
1. palpable
-autoimmune vasculitis

  1. non-palpable
    -immune thrombocytopenic purpura

FRACP Clinical Exam (14 decks)

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