Summary Book Haematology Flashcards

1
Q

Presentation of Multiple Myeloma

A

CRAB = bone pain / fracture (60%), anaemia (normochromic normocytic 70%), renal impairment (casts from light chains, stones - calcium, 50%), hypercalcaemia (30%). Recurrent infections due to dysfunctional immunoglobulins. Bleeding - platelet dysfunction and Ig inactivation of procoagulant. Hyper viscosity - especially IgM (waldenstroms). Systemic AL amyloid. Complications - weight loss, CKD, fracture, anaemia, death.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Signs of multiple myeloma

A

weight loss, anaemia, bone tenderness, splenomegaly, signs of infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Investigations for multiple myeloma

A

FBP, blood film, UEC, LFT, quantitative serum immunoglobulin (increased IgG or IgA), kappa/lambda light chain ratios + light chain in urine (bence jones proteinuria), high b2 macroglobulin and low albumin for staging, BMAT, skeletal survey

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Management of multiple myeloma

A
  1. Bortezanib + Thalidomide + Dexamethasone induction. Thalidomide and dexamethasone maintenance. 2. Daratumumab (anti CD38). 3. Zoledronic Acid (bone protection + prevent hypercalcaemia). 4. EPO for anaemia. 5. Radiotherapy for bone lesion + preventative joint replacement. 6. Transplant if less than 70 years old with minimal comorbidities.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Symptoms of chronic myeloid leukaemia

A

B symptoms, lymphadenopathy, infections, abdo fullness and splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Diagnosis of chronic myeloid leukaemia

A

Blood tests = basophilic/eosinophilic, Philadelphia chromosome (BCR-ABL t(9:22)). Blast phase occurrence (>20 blasts). Family history.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Signs of chronic myeloid leukaemia

A

splenomegaly, anaemia, complications of JAK2 inhibitors (fluid retention, CCF, VTE, myelosuppression, hypothyroid, neuropathy)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Management of chronic myeloid leukaemia

A

Tyrosine kinase inhibitors - 1st line = Imatinib, Rusolitinib. Allogenic stem cell transplant if not responsive to TKI.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Diagnosis and management of essential thrombocytopenia

A

Platelets >800. Conduct investigations to exclude infection, malignancy, inflammation, bleeding, recent surgery. Most require no treatment, however neurological symptoms may prompt use of aspirin. Use hydroxyurea if not responding to aspirin.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Primary and secondary causes of polycythaemia

A

Primary = underlying proliferative disorder (PRV, ET, MF, CML). Secondary causes = increased EPO, smoking, COPD, OSA, phaeo, cushings.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Complications of polycythaemia

A

stroke, IHD, PVD, abdo clots

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Signs of polycythaemia

A

plethora, cushings, clubbing, PVD, central cyanosis (congenital heart disease), splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Management of polycythaemia

A

JAK2 testing. FBP with increase Hb/Hcrit. Exclude secondary causes. Aspirin and venesections (aim HCT <40), add hydroxturea if >60 years old or history of VTE.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Complications associated with thrombophilia

A

clot, VTE risk, anticoagulants, previous miscarriage (anti-phospholipid syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Signs of thrombophilia

A

obese, immobile, chronic venous insufficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Investigations for thrombophilia

A

Thrombophilia investigations. APS Ab, Factor 5 leiden, anti-thrombin 3, protein c/s deficiency, prothrombin gene mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Management of thrombophilia

A

Most get NOACs, even cancer patients can have Rivaroxaban. Warfarin if mechanical valves.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Compare key associations of hodgkin and non-hodgkin lymphoma

A

Hodgkin - localised nodes, reed steinberg cells, EBV, B symptoms. Non-hodgkin - multiple nodes, B cells, HIV and autoimmune.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Poor prognostic factors for lymphoma

A

age over 60, raised LDH, low ECOG, stage 3 or 4, extranodal involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are the 4 stages of lymphoma

A
  1. local. 2. distal LN above diaphragm. 3. below diaphragm LN. 4. end organ.
21
Q

Complications of lymphoma

A

Acute secondary to treatment = febrile neutropenia, nausea, infections. Secondary malignancy (lung / breast / skin) = from chemotherapy or chest radiotherapy. Secondary cardiac (anthracycline) or respiratory (bleomycin) complications

22
Q

Management of hodgkin lymphoma

A

ABVD (+/- radiotherapy) or BEACHOP (85%)

23
Q

Management of non-hodgkin lymphoma

A

DLBCL = RCHOP, hyper CVAD. Follicular = R-CVP, R-bendamustine. MALT = treat H. pylori.

24
Q

Investigations for lymphoma

A

core biopsy, bloods, PET, BMAT, ana arbor staging

25
Q

Risk factors for lymphoma

A

EBV, HIV, HHV8, HTLV-1, transplant (immunosuppressant = PTLD)

26
Q

Presentation of lymphoma

A

B symptoms, rubbery painless lymphadenopathy, pruritis, dyspnoea if mediastinal mass, organomegaly, possible anaemia/infection

27
Q

Presentation of myelofibrosis

A

Asymptomatic found on routine FBC, splenomegaly

28
Q

Non-malignant and malignant causes of myelofibrosis

A

Non-malignant = SLE/HIV/TB/hyperparathyroidism/renal osteodystrophy. Malignant = CML/acute leukaemia/lymphoma/multiple myeloma/metastatic carcinoma

29
Q

Investigations for myelofibrosis

A

Blood film with poikilocytosis and leucoerythroblastic changes, bone marrow biopsy, JAK2 V617F

30
Q

Management of myelofibrosis

A

Supportive with transfusions, hydroxyurea if symptomatic with organomegaly or thrombocytosis

31
Q

Indications for stem cell transplant

A

acute leukaemia (AML), CML, lymphoma, aplastic anaemia, multiple myeloma

32
Q

Investigations pre stem cell transplant

A

bone marrow biopsy, HLA typing

33
Q

Important parts of management of stem cell transplant recipients

A

Preventative antibiotics, ATG or immunosuppressants (cyclosporin, tacrolimus, MMF) for GVHD, steroids for acute GVHD, supportive care with blood products, antibiotics for febrile neutropenia, vaccines for first 6 months post transplant

34
Q

5 year survival for types of leukaemia - NHL, CML, AA

A

NHL - 50%, CML - 70%, AA - 90%

35
Q

Peri-transplant complications of stem cell transplant

A

infection, mucositis, acute GVHD, bleeding, veno-occlusive disease

36
Q

Post-transplant complications of stem cell transplant

A

chronic GVHD, CMV/BK/fungus, poor engraftment (check chimerism), relapse, secondary malignancies

37
Q

Organs involved in acute vs chronic GVHD

A

acute = skin, intestines, liver. chronic = eyes, lungs

38
Q

Examination of stem cell transplant recipient

A

GVHD signs, veno-occlusive disease = hepatomegaly and ascities, skin malignancy / infection

39
Q

Diagnosis of AML

A

blast >20 in peripheral blood with Auer rods, CD13/33 positive

40
Q

Compare good and prognostic factors for AML

A

AML poor prognosis = >60yo, FLT3 mutation, TP53 mutation, delete chromosome 5 or 7, t(9:22) - philadelphia. AML good prognosis = T(15:17) - APML, inversion 16, NPML 1

41
Q

Causes of MCV <80

A

Fe def, lead, thalassemia, chronic disease

42
Q

Causes of MCV 80 to 100

A

bleeding, haemolysis, marrow failure, chronic disease

43
Q

Causes of MCV >100

A

B12 or folate def, drugs, reticulocytes, liver disease, hypothyroidism, myelodysplasia

44
Q

Symptoms and signs of haemolytic anaemia

A

Symptoms of anaemia = lethargy, dyspnoea, chest pain. Previous haemolysis / hereditary disorders = spherocytosis, sickle cell, 6GDP def, thalassemia. Lymphoma / SLE (warm AIHA). Infections (cold AIHA from mycoplasma, DIC in sepsis, HUS from Ecoli). Neurological symptoms (thrombotic thrombocytopenic purpura, spherocytosis, sickle cell). Connective tissue disease (sclerosis, SLE). Other signs - pallor icterus, scars of heart valve surgery, splenomegaly, lymphadenopathy, chronic liver disease, infections.

45
Q

Investigations for haemolytic anaemia

A

FBC, Fe, B12, folate, haemolysis screen (LDH, bili, reticulocytes, haptoglobins, coombs test = if positive then antibody cause), blood film (schistocytes, spherocytes, sickle cell, red cell agglutin - fragmentation), scope if GI loss, EPO if CKD, haemoglobin subclasses (G HbA, HbA2, HBF).

46
Q

Management of warm AIHA

A

steroids, splenectomy, IVIG, rituximab

47
Q

Management of TTP

A

plasmapheresis

48
Q

Management of aHUS

A

Eculizumab

49
Q

Complications of BMAT

A

General = sepsis, skin cancer, headache (PRES). Infections may be associated with febrile neutropenia. Early complications consider mucostis, infection, graft failure, immunodeficiency, veno occlusive disease or acute GVHD. Late complications consider chronic GVHD, gondal toxicity and neurological.