Haematology Long Case Flashcards
1
Q
Haemolytic anaemia (History)
A
- Risk factors
- family history
- connective tissue dx
- lymphoma or malignancy
- mechanical valve replacement
- malignant hypertension
- medications - Presentation
- prodrome (infections)
- symptoms of anaemia
- acute sickle crises
- neurological symptoms
- renal failure - Investigations
- FBE, MCV, film
- UEC
- LFTs
- reticulocyte
- biliriubin
- LDH
- serum haptoglobin
- urinary haemosiderin
- Coomb’s test
- CD55/59 - PNH
- Hb electrophoresis
- red cell membrane study (5-EMA)
- G6PD assay - Treatment
- remove underlying cause
- avoid precipitants
- supportive care
- steroids
- folate supplementation
- splenectomy
- azathioprine, cyclophosphamide
- rituximab
- IVIG
- consider transfusion
- plasma exchange
- eculizumab (PNH, aHUS)
2
Q
Splenectomy advice
A
- Vaccinate for pneumococcus, HiB, meningococcus, influenza
- Alert bracelet
- Prophylactic antibiotics
- Treat animal bites aggressively
- Treat septic patients with empirical cover for encapsulated organisms
3
Q
Haemolytic anaemia (Examination)
A
- Frontal bossing, maxillary marrow hyperplasia
- Pallor, icterus
- Lymphadenopathy
- Iron overload - skin pigmentation, cardiac failure, hepatomegaly, features of chronic liver disease
- Valve replacements, AS
- Splenomegaly
- focal neurological deficits
- Joint swelling
- Leg ulceration
- Urinalysis
4
Q
Thrombophilia (History)
A
- Risk factors
- family history
- smoking, OCP, malignancy, immobility, surgery - Presentation
- arterial or venous
- recurrent miscarriages - Investigations
- FBC and ESR
- antithrombin III deficiency
- protein C & S deficiency
- Factor V leiden
- prothrombin gene mutation
- anti phospholipid antibodies
- homocysteinuria
- PNH (flow cytometry)
- Plasma homocysteine - Management
- identify risk factors
- anticoagulation
- if warfarin - target INR, logistics - Complications
- post-thrombotic syndrome
5
Q
Thrombophilia (Examination)
A
- Obesity
- Venous insufficiency
- Peripheral vascular disease
- Features of underlying myeloproliferative dx, SLE, nephrotic syndrome, cardiac failure or malignancy
6
Q
Thrombophilia (Indications for testing)
A
- Recurrent venous thrombosis
- Venous thrombosis < 45
- Thrombosis at unusual site
- portal vein
- cavernous sinus
- hepatic vein - Family history
7
Q
Polycythaemia (History)
A
- Risk factors/aetiology
- primary (JAK2 V617F)
- secondary (increased EPO or hypoxic states) - Presentation
- vascular thromboses
- abdominal pain
- aquagenic pruritus
- gout
- symptoms of underlying dx - Investigations
- FBE, haemotocrit
- Abdominal US
- EPO level
- ABG
- renal disease
- JAK2 kinase mutation - Management
- Phlebotomy (Hct <0.45)
- Aspirin
- Consider hydroxyurea
- Anti-histamines
- Treat gout
- Treat underlying disease - Complications
- Outlook
8
Q
Polycythaemia (Causes)
A
- Primary (PCV - JAK2 mutation)
- splenomegaly
- normal PaO2
- normal EPO/or low - Secondary
- increased EPO
- - RCC
- - hepatoma
- - Cushing’s
- - PCKD
- - virilizing syndromes
- hypoxic states
- OSA
- congenital cyanotic heart dx
- chronic lung disease
- CO poisoning
- smoker
- Relative
- stress
- dehydration
9
Q
Polycythaemia (Exam)
A
- Plethora/cyanosis/hydration
- Blood pressure
- Cushingoid features
- Signs of gout
- Signs of PVD
- Cardio/respiratory dx
- Hepatosplenomegaly
- Renal masses
- Scratch marks
- Urinalysis
10
Q
Idiopathic myelofibrosis (History)
A
- Risk factors/aetiology
- idiopathic myelofibrosis
- secondary myelofibrosis (SLE, HIV, TB, CML, leukaemia, lymphoma, multiple myeloma, metastatic carcinoma) - Presentation
- splenomegaly
- extra-medullary haematopoiesis - Investigations
- blood film
- bone marrow biopsy - Management
- supportive
- blood transfusions
- hydroxyurea
- folate/B12
- ruxolinitib (JAK2 inhibitor)
- allogeneic transplant
11
Q
Essential thrombocythaemia (History)
A
- Risk factors/aetiology
- primary
- secondary (iron deficiency, infection, bleeding, asplenia, inflammation, malignancy, polycythaemia, CML) - Presentation
- digital ischemia
- TIA, visual disturbances
- VTE
- bleeding
- erythromelalgia - Investigations
- FBE
- JAK2 mutation
- exclude CML (Ph chromosome, BCR-ABL) - Management
- observation
- aspirin
- hydroxyurea
12
Q
CML (History)
A
- Aetiology
- BCR-ABL from Ph chromosome (t 9;22) - Presentation
- splenomegaly
- fatigue, malaise, weight loss
- progression (fever, infections, bone pain, haemorrhage/thromboses) - Investigations
- WCC > 50, differentials (basophils/eosinophils)
- Blasts
- Ph chromosome - Management
- imatinib (rash, GI, oedema)
- dasatinib (pleural effusion)
- nilotinib (cardiovascular)
- monitor with BCR-ABL quantitative assay
- allogeneic bone marrow transplant - Complications
- blastic transformation
13
Q
Lymphomas (History)
A
- Aetiology
- Non Hodgkin’s lymphoma
(DLBCL, CLL, follicular)
- Hodgkin’s lymphoma
- risk factors (HIV, immunodeficiency, immunosuppression, autoimmune disease) - Presentation
- lymphadenopathy
- extra-nodal (GI, skin, neurological)
- B symptoms
- bony pain, infection, haemorrhage
- ECOG status - Investigation
- lymph node biopsy (excisional)
- PET/CT, LFT
- ESR, reversed CD4/CD8
- MRI +/- LP (CNS involvement) - Management
- based on staging (Ann Arbor for Hodgkin’s)
- chemotherapy (ABVD - adriamycin, bleomycin, vinblastine, dacarbazine)
- radiotherapy - Complications
- mantle radiotherapy (pneumonitis, hypothyroidism, pericarditis, myocardial fibrosis, spinal cord injury) - Outlook
- prognosis (age, LDH, Ann Arbor staging, extra-nodal, ECOG)
- impact (depression, coping mechanism, dependents, social support, work)
14
Q
Multiple myeloma (History)
A
- Aetiology (MGUS, smouldering myeloma)
- risk factors - age/exposure - Presentation
- hypercalcaemia
- renal disease
- anaemia
- bone pain/fractures
- infections
- bleeding
- amyloidosis - Investigations
- FBE - normocytic anaemia
- ESR
- SPEP, SFLC, UPEP
- Calcium
- renal function
- serum albumin
- B2 microglobulin level
- skeletal survey
- bone marrow biopsy (>10%) - Management
- bisphosphonates
- treat complications
- autologous bone marrow transplant
- chemotherapy (VCD) - Complications
- side effects of treatment (peripheral neuropathy, thrombosis) - Outlook
15
Q
Bone marrow transplantation
A
- Underlying disease indication
- Transplantation (autologous or allogeneic)
- ablation method
- harvesting method
- donor details (HLA match) - Perioperative course
- duration
- sepsis, pancytopenia, mucositis - Surveillance/investigations
- rejection
- chimerism (day 100) - Management
- irradiated transfusions
- GCSF
- GVHD prophylaxis (prednisolone, MTX, cyclosporin)
- GVHD treatment (prednisolone, ATGAM, etc)
- Bactrim, posaconazole, valganciclovir
- ursodeoxycholic acid/TPA (liver) - Early complications of management
- acute GVHD 3/12 (sensitization/match)
- rash, mucositis, cystitis, pneumonitis, pulmonary oedema, renal failure)
- infection
- immunodeficiency
- veno-occlusive disease of liver - Late complications of management
- infertility
- neurological
- chronic GVHD (>3/12)
- infection
- relapse of treated condition
- second malignancy
- immunodeficiency - Outlook/impact
- prognosis (better with non-malignant indication/autologous)
- QOL
- coping/support
