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Hark and Paul's clinical review > Haematology > Flashcards

Haematology Flashcards

1
Q

What are the late complications of BMT?

A
  • treatment related: gonadal toxicity, neuropathy
  • chronic GVHD
  • infection
  • relapse
  • secondary malignancy
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2
Q

What are signs of acute GVHD?

A

Skin: maculopapular rash
GI: diarrhea, N/V
Liver: deranged LFT/bili, hepatomegaly

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3
Q

What is the treatment of acute GVHD?

A
  • topical steroid
  • systemic steroids: methylpred
  • octreotide for diarrhea
    Second line agents: MMF, etanercept
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4
Q

What are the features of chronic GVHD?

A 
> 100 days
Skin: scleroderma-like
GI: dry oral mucosa with ulceration a, dysphagia, chronic diarrhea, malabsorption
Lung: bronchiolitis obliterans
Blood: thrombocytopenia
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5
Q

What is the treatment of chronic GVHD?

A
  • enrol in clinical trial
    Otherwise:
  • prednisone
  • add CNI if no improvement after 4-6/52 of pred
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6
Q

What is the genetic abnormality in CML?

A

Bcr-ABL - always on tyrosine kinase

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7
Q

What is the treatment of CML?

A

Stem cell tx (may be curative)
TKI: imatinib
Chemotherapy for palliation

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8
Q

What are the B symptoms?

A

Fever
Wt loss
Night sweats

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9
Q

What are reed sternberg cells associated with?

A

Hodgkin lymphoma

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10
Q

What is the management of Hodgkin Lymphoma?

A
  1. RTx

2. Chemo: ABVD, BEACOPP

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11
Q

What is the management of NHL?

A

High grade: Rituximab-CHOP

BMTx

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12
Q

What are the complications of lymphoma treatment?

A
  • effects of radiation: fibrosis, pericarditis
  • secondary malignancies: leukaemia, breast, skin
  • fertility: reduced, teratogenic effects of chemo
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13
Q

What causes microcytic anemia?

A
  • iron deficiency
  • thalassemia
  • anemia of chronic disease
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14
Q

What causes normocytic anemia?

A
  • acute bleeding
  • bone marrow failure: aplastic, mds, infiltrative disease
  • CKD
  • hemolysis
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15
Q

What are the causes of macrocytic anemia?

A
  • B12 or folate deficiency
  • alcohol
  • liver cirrhosis
  • mds
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16
Q

What investigations for anemia?

A
  • FBC, Blood film
  • reticulocytes: low if BM issue, high if loss/destruction of RBC
  • iron studies
  • b12/folate
  • intrinsic factor ab
  • hemolysis: low haptoglobin, raised bili, DAT
  • BM biopsy
  • TFT
  • electrophoresis for hemoglobinopathies
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17
Q

What are the clinical features of MM?

A 
CRAB:
- hypercalcemia
- renal failure
- anemia
- bone pain
Also, fractures, amyloidosis, cord compression
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18
Q

What investigations for MM?

A
  • FBC, ESR, CMP, EUC
  • serum and urine EPG/IEPG: monoclonal protein
  • serum free light chains
  • B2 microglobulin for prognostication
  • urine bence Jones
  • bone marrow biopsy
  • XR skeletal survey
  • MRI if concern for spinal cord compression
  • urate
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19
Q

What is the treatment for MM?

A 
Chemotherapy
- high dose steroid plus thalidomide/lenalidomide
- bortezomib
- cyclophosphamide
ATSCTx
Symptom control:
- RTx to bony lesions, bisphosphonates
- allopurinol for raised urate from treatment
20
Q

What are the prognostic factors in MM?

A
  • 13q deletion
  • high b2 microglobulin
  • poor ECOG
  • anemia
  • hypercalcemia
  • renal failure
21
Q

What are the early complications of BMTx?

A
  • Infection
  • Graft Failure
  • acute GVHD
22
Q

What are indicaitons for BMTx?

A

1) Brief if long time ago, only spend more time if a) recur b) relevant now
2) What?
3) How presented? (brief) = e.g. “Allogenic BMT done for AML for which he presented with
pancytopenia and Hb of 60”
4) Risk profile - cytogenetics if relevant
5) Mx prior to Tx (brief) - mention relevant chemos if going to have long term SEs e.g:
1) Anthracyclines: cardiac toxicity
2) Vinca alkaloids: neuropathy
6) Donor? Well matched?
7) Blood group
8) CMV status
9) Recipient conditioning - myeloablative chemo/ full body radiation

23
Q

What are early complications of BMTx?

A

1) Induction related: mucositis, cystitis, interstitial pneumonitis (usually CMV), renal impairment
2) Graft failure
3) Infection
4) Immunodeficiency
5) Acute GVHD (within 3/12) - rash (can be blistering), GI (similar to crohn’s), Liver function derangements
6) VOD (veno-occlusive disease - sinusoidal obstructive disease of the liver - typically within 1 month, presents with triad of raised Bili (jaundice), fluid retention (ascites) and tender hepatomegaly. Treatment: Defibritide

24
Q

What are late complications of BMTx?

A

1) Treatment related: Gonadal toxicity, Cataracts, Neurological
2) Chronic GVHD (> 100 days) - rare to persist >3 yrs
- Sicca syndrome (like Sjogren’s) = dry eyes, mouth (xerostomia), leading to ulceration, pain and poor dentition. Needs intensive topical tx (steroid drops)
- Skin: patchy skin disease. Most feared form resembles SSc (sclerodactyly, restricted movement, reduced chest expansion)
- Lung: obstructive pattern- Bronchiolitis obliterans, chronic cough, SOB, wheeze; Fatal if progressive
- GI: milder IBD symptoms
- Abnormal LFT - classically mixed pattern: ↑ALT, ↑GGT
3) Infection
4) Status/Relapse of original treated condition
- Remission? result of latest PET/ bx?
5) Second malignancy

25
Q

Infections and prophylaxis post BMTx?

A

1) More common post BMT than solid organ
2) Prophylaxis with Valaciclovir, TMP-SMX and Fluconazole for 6 months
3) Post transplant, functionally hyposplenic - may give prophylaxis against encapsulated bacteria, ensure vaccinations up to date (childhood vax no longer applies)

26
Q

Drug SEs post BMTx?

A

Post BMTx Drug SEs: Steroids, CNI, MMF

  • Usually weaned off immunosuppression by 6 months
  • Suggestive of GVHD if still on > 6 months
27
Q

Very Late SEs (> 3 years) post BMTx?

A

1) Malignancy risk
2) Endocrine - thyroid, hypogonadism, infertility
3) Vision
4) Psychiatric - depression, effect on work, function

28
Q

What is the management of Haemophilia A?

A

“A and B involve 8 and 9; FHx 70%; Sporadic 30%”

  • Avoid Aspirin & NSAIDs (NSAIDS inhibit platelets, although COX-2 inhibitors celecoxib platelets are spared)
  • DDAVP = tx of choice in mild-­‐moderate haemophilia
  • Recombinant FVIII/ FVIII concentrate (regular prophylaxis if severe disease c frequent bleeds)
  • Dose of recombinant factor 8 = 15 - 30 units/ kg
  • FFP/ CPP
  • Dental care (Aim to avoid need for extractions)
  • Restrictions re: contact sports
  • Travel issues (FVIII supply, equipment etc)
29
Q

What is the management of Haemophilia B?

A

“A and B involve 8 and 9; FHx 70%; Sporadic 30%”

  • CHRISTMAS DISEASE FIX deficiency/ dysfunction
  • X­‐linked recessive; 1/100,000 males affected
  • Clinically indistinguishable from Haemophilia A
  • Management: FFP Recombinant FIX
  • Haem B - longer half life of recombinant factor 9
  • NB: Don’t use Prothrombinex to due thrombotic risk.
30
Q

What causes recurrent bleeds in Haemophilia on prophylactic factors?

A
  • Prophylactic factors to maintain basal level ~ 1% (makes spontaneous bleeds less likely)
  • Typically dosed 3 times a week, half life of recombinant factor about 12 hrs
  • If recurrent bleeds - think of inhibitors developing. Very difficult to treat. Usually develop within first 50 treatment days. Use bypass agents, but they are less effective
31
Q

What are late complications of Haemophilia?

A
  • Arthropathy - joint destruction, marked thickening and proliferation of synovium. MRI useful for diagnosing this. May require synovectomy
  • Venous access may become an issue.
  • New treatments coming through are subcut injections
32
Q

=========================
Anaemia - categorised by cell size

What are the causes of Microcytic anaemia?
What are the causes of Macrocytic anaemia?
What are the causes of Normocytic anaemia?

A

> Microcytic - Mnemonic “Small Typically Iron”
-Sideroblastic
-Thalassaemia (usu high RBC count >5Bill/L)
-Iron deficiency
Macrocytic - “My Blood Has Large Erythrocytes”
-Myelodysplasia (normoblastic, round macrocytes)
-B12/fol deficiency (megaloblastic, with oval macrocytes and hypersegmented neutrophils; failed DNA synth; likely if MCV >110fL)
-Haemolysis; Hypothyroidism (macro or normocytic)
-Liver disease
-Embryo (pregnancy)
Normocytic (76-96fL) - “Exclude Chronic Anaemia”
-Endocrine (hypopit, hypothyroid, adrenal)
-Combined deficiency; Chronic disease (cancer, rheumatic disease)
-Acute blood loss/Aplastic

33
Q

Anaemia

What are the causes of congenital haemolytic anaemia?

A

> Mnemonic: “MEH” Membrane/Enzyme/Haemoglobin
-Membrane: Spherocytes (hered spher. most common, and is auto dominant), Elliptocytosis
-Enzyme: G6 PD (Glucose 6 Phos dehyd) deficiency - it maintains important antioxidant glutathione
-Haemoglobin: Thalassaemia, Sickle Cell Disease
Presentation: +ve FHx, anaemia, splenomegaly, jaundice
Risks/precipitants: infection, oxidation from drugs like dapsone, quinine and nitrofurantoin
Ix: film for spherocyte, elliptocyte,

34
Q

What are the natural anticoagulants?

A

> Mnemonic: “AP”TT
Anti-thrombin III
-Can be deficient in nephrotic syndrome; get arterial and venous thrombosis
Protein C + S
-Factor V Leiden mutation is auto-dominant, and prevents Protein C/Factor V from stopping the clotting cascade
-Protein C/S deficiencies are less common

35
Q

What are the causes of excessive bleeding?

A

> Capillary/Platelet/Coagulation
Capillary
-Inherited: collagen diseases (such as Ehlers Danlos); Hereditary haemorrhagic telangiectasia
-Acquired: severe infection, purpuras- senile, steroid, Henoch-Schonlein
Platelet
-ITP: young females +/- splenomegaly
-Marrow infiltration (secondaries, leukaemia)
-Marrow aplasia (drugs, viral)
Coagulation
-Anticoagulant treatment
-Haemophilia A or B (Haemoph.A is x-linked recessive of factor 8; Haemoph.B lack factor 9)
-Von Willebrand’s disease (most common bleeding disorder in the west; auto dominant; adhesion of platelets to damaged vessel walls; bruising menorrhagia and epistaxis)

36
Q

What are the causes of severe eosinophilia?

Causes of severe eosinophilia with abnormal CXR?

A

> Severe Eos. (>5 bil/L)
-mnemonic “Painful Blisters”
-Parasitic infection (not common in the west)
-Blistering skin infections (Pemphigus, pemphigoid, eryth. multiforme)
Severe Eos. + abnormal chest infection
-mnemonic “High Eosinophils + Lung Trouble”
-Hypereosinophilic syndrome (eos., HFPEF, hepatosplenomegaly)
-Eosinophilic pneumonia
-Leukaemia (eosinophilic)
-Tropical pulmonary eosinophilia
Less Severe Eosinophilia (0.5-2 bil/L)
-asthma, rhinitis, drug allergies (sulfonamides, nitrofurantoin), Churg-Strauss, Hodgkin’s lymphoma

37
Q

What are the causes of high ESR >100?

What is the differential for PMR?

A

> Mnemonic “Vasculitis May Prolong Sedimentation”

  • Vasculitis
  • Myeloma (disease of elderly; high immunoglobulins; risk of arterial and venous thrombosis)
  • Polymyalgia Rheumatic (pain and stiffness in shoulder and pelvic girdle, rare <50yrs, associated GCA, muscle weakness is NOT a feature- if this is present suspect polymyositis; treatment is with high dose steroids)
  • Sepsis
38
Q

What are the common causes of immunodeficiency?

A

“Drugs May Cause Lowered Immunity”

  • Drugs- phenytoin, penicillamine can induce hypogamma
  • Myeloma- high levels of monoclonal gamma-globs have an immunoparetic effect, suppressing body’s immune response to antigens (note the immunosuppressive effect of immunoglobulins is used to suppress autoimmune response in conditions like GBS)
  • Common Variable Hypogammaglobulinaemia - this is primary; the rest secondary
  • Lymphoma/Leukaemia (CLL)- associated with hypogamma
  • Infection (HIV) impairs cell-mediated (CD4 T) immunity;
  • (nephrotic syndrome also loses gammaglobulins)
39
Q

Which form of leukaemia is most common in children?

A

Mnemonic “ALL the kids respond”
>Acute Lymphoblastic (ALL) most common in children
-more than 90% respond; and 70% cured
-acute associated with blasts, and rapid progression
-WCC may be raised/normal/low; the blasts are key
-AML vs ALL - AML have Auer rods- small inclusion bodies
-present with bone marrow failure: infection, anaemia, bleeding
>CML- suspect if high WCC (typical>50 neutrophils)
>CLL- suspect if high WCC (typical>15 lymphocytes)

40
Q

What is the blood film and presentation of CLL?

A

Mnemonic CLL is the “B disease”
-B lymphocytes (95%)- initially just high WCC
-Bone marrow failure (anaemia, infection, bleeding)
-Bleeding
-Broken cells (smear cells)
>M: chlorambucil (1st line)
- treat folate deficiency (high turnover)

41
Q

What is the onset, blood film and pathology of CML?

DDx for splenomegaly and myeloid precursors?

A

CML “Myeloid Leukaemia Patient”
-Middle age
-Leukoerythroblastic picture (myeloid precursors, and nucleated RBCs)- ddx = marrow infiltrate or severe stressors or sepsis
-Philadelphia chromosome +ve (almost all; translocation of long arms t(9:22) chromosomes)
>DDx for splenomegaly and myeloid precursors?
-myelofibrosis: Philadelphia -ve (and high NAP neutrophil alk phos score)

42
Q

Microangiopathic haemolytic anaemia

What are the causes of MAHA?

A

“Ma H A Causes Blood Vessel Damage”
-Mucinous adenoca
-HUS/TTP and aHUS (haem uraemic synd; thrombotic thrombocytopaenic purpura; HUS is likely in E.Coli O157:H7; shiga toxin, gastro with fever and renal impairment; TTP due to ab to ADAMS which drops <10%; if ADAMTS13 levels >10% and AKI, then likely aHUS- check c3,c4,factor H, I; aHUS responds to eculizumab; factor H may respond to plasmapharesis and immunosuppresives)
-Accelerated HTN
-Connective tissue disease
-Burns
-Vasculitis
-DIC Disseminated Intravascular Coagulation
>Features: fragment (helmet) cells, schistocytes

43
Q

What are the causes of pancytopaenia?

Causes of pancytopaenia with raised MCV?

A

> Pancytopaenia: “My Spleen Injures Cells”

  • Marrow infiltration
  • Spleen (hypersplenism)
  • Indiopathic acquired
  • Congenital (Fanconi’s anaemia - auto recessive, bone marrow failure, developmental abnormalities, reduced life expectancy)

> Pancytopaenia/High MCV:

  • “Impaired Marrow Produces Some Big Cells”
  • Infections EBV
  • Myxoedema/Myelodysplasia
  • PNH Parox nocturnal haemoglobinuria
  • SLE
  • B12
  • Cytotoxics
44
Q

What are the causes of polycythaemia?

A

> “Primary/Secondary/Inappropriate”

  • Primary: Polycythaemia rubra vera (independent of EPO; typically raised platelets and neutrophils unlike the other causes)
  • Secondary: Chronic lung disease, R-to-L cardiac shunts often congenital, High altitude (hypoxia=> elevated EPO)
  • Inappropriate(Tumours): renal, liver brain (elevated EPO without hypoxic stimulation)
45
Q

What are the causes of thrombocytosis?

A

> Mnemonic: “Massive Bleeding Increases Platelet Count Significantly”

  • Myeloproliferative disease
  • Bleeding (transient)
  • Inflammation (transient) / Iron deficiency (reduced MCV)
  • Primary thrombocytosis
  • Connective Tissue disease/Cancer
  • Splenectomy (see Howell-Jolly bodies)
46
Q

What are conditions that cause both arterial and venous thrombosis?

A

> Mnemonic “Pretty Sticky Blood Thromboses All Vessels”

  • PNH (membranes lysed by complement; dark urine and thrombosis; haemolytic anaemia)
  • Sickle Cell
  • Behcet’s syndrome (orogenital ulceration; pathergy is papules >2mm at trauma e.g. injection sites)
  • Thrombophilia
  • Antiphospholipid syndrome (clots, miscarriage and livedo reticularis)
  • Vasculitides
  • (homocystinuria)
47
Q

Which are the vitamin K dependent factors?
What is the half life of heparin?
How common is heparin induced thrombocytopaenia?

A
  • vitamin K dependent factors: 2/7/9/10
  • heparin half life - 2 hrs; APTT (not useful for LMWH which is predictable and does not raise APTT- is monitored with anti Xa if long term use)
  • HIT occurs in 1-5%; subsequently one third of them may suffer from arterial and/or venous thrombosis (test AntiPF4 platelet factor 4)

Hark and Paul's clinical review (12 decks)

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