Clinical Haematology/Oncology Flashcards
Features of G6PD deficency
Neonatal jaundice Intravascular haemolysis Gallstones Splenomegaly Heinz bodies
Drugs inducing haemolysis in G6PD
Anti-malarials: primaquine
Ciprofloxacin
Sulphonamides, sulphasalzine, sulfonylureas
Raised leucocyte alkaline phosphatase
Myelofibrosis Leakaemoid reactions Polycythaemia rubra vera Infections Steroids Pregnancy OCP
Cyclophosphamide; MoA
Causes cross linking in DNA
Cyclophosphamide: SE
Haemorrhagic cystitis
Myelosuppression
Transitional cell carcnioma
Bleomycin: MoA
Degrades performed DNA
Bleomycin; SE
Lung fibrosis
Anthracyclines; MoA
Stabilises DNA complex
Inhibits DNA and RNA synthesis
Anthracyclines; SE
Cardiomyopathy
Methotrexate: MoA
Inhibits dihydrofolate reductase
Methotrexate: SE
Myelosuppression, mucositis, liver fibrosis, lung fibrosis
Flurouracil; MoA
Pyrimidine analogue inducing cell cycle arrest and apoptosis
Flurouracil; SE
Myelosuppression, mucositis, dermatitis
Vincristine, vinblastine; MoA
Inhibits microtubule formation
Vincristine; SE
Reversible peripheral neuropathy, paralytic ileus
Vinlastine; SE
Myelpsuppression
Cisplastin; MoA
cross linking in DNA
Cistplastin; SE
Ototoxicity, peripheral neuropathy, hypo Mg
Hereditary angioedema; causes
AD condition assoicated with low plasma levels of C1 inhibitor
Hereditary angioedema; screening
Serum C4
Hereditary angioedema; features
Painful macular rash
Painless, non-pruritic swelling of SC tissues
Hereditary angioedema; acute Mx
IV C1-inhibitor concentrate, FFP
TTP; what?
Abnormally larger and sticky multimers of VWB factor causing platelet aggregation
TTP; features
Fever, neuro signs, microangiopathic haemolytic anaemia, thrombocytopenia, renal failure
Methaemoglobinaemia; what?
Haemoglobin which has been oxidised from Fe2+ to Fe3+
Methaemoglobinaemia; acquired causes
Drugs; sulphonamides, nitrates, dapsone, sodium nitroprusside, primaquine
Methaemoglobinaemia; features
‘chocolate’ cyanosis, dyspnoea, anxiety, headache, acidosis, arrhythmias, seizures, coma, normal pO2 but decreased saturations
CML; genetic associations
Philadelphia chromosome
Translocation between long arm of chromosome 9 and 22 (t9:22)(q34; q11)
CML; managment
Imatinib
Imatinib; MoA
Tyrosine kinase inhibitor
Acute promyelocytic leukaemia M3; what?
associated with t(15;17)
Fusions of PML and RAR-alpha genes
AML; film
Auer rods
Cryoglobulinaemia; Type 1
Monoclonal; IgG or IgM
Associations; MM, Waldenstrom macroglobulinaemia, Raynauds
Cryoglobulinaemia; Type 2grens
Mixed monoclonal and polyclonal
Usually with Rh factor
Associations; HCV, RA, Sjogren’s, lymphoma
Cryoglobulinaemia; Type 3
Polyclonal
Associations; RA, Sjo
vWB; what?
AD bleeding disorder
vWB; Ix
Prolonged bleeding time
APPT
Reduced VIII
vWB; Mx
Tranexamic acid
Desmopressin
Paroxysmal nocturnal haemoglobinuria; what?
Acquired disorder leading to intravascular haemolysis
Paroxysmal nocturnal haemoglobinuria; features
Haemolytic anameia
Pancytopenia
Haemoglobinuria
Thrombosis
Paroxysmal nocturnal haemoglobinuria; Ix
Flow cytometry of blood to detect CD59 and CD55
MGUS; what?
Common condition causes paraproteinaemia
MGUS; difference from myeloma
Normal immune function
Normal beta-2 levels
Lower and stable paraproteinaemia
No CF of myeloma
B Cell deficinecy
Shitty B Cells
- selective IgA deficinecy
- Burton’s
- CVID
Combined B & T cell deficnecy
WASH your Bs and Ts
- Wiskott Aldrichs
- Ataxic telangectasia
- SCID
- Hyper IgM
Selective Oestrogen Receptor Modulators
Tamoxifen
Used is oestrogen +ve breast ca
Aromatase inhibitors
Reduce peiperhal oestrogen synthesis
Anastrozole and letrozole
Used in ER + breast ca
Waldenstrom’s macroglobulinaemia; features
Monoclonal IgM paraproteinaemia Hyperviscosity HSM LN Cryoglobulinaemia
Tumour lysis syndrome; Rx
Rasburicase
Rasburicase; MoA
Metabolises uric acid to allantoin
Intravascular haemolysis
Mismatched blood transfusion G6PD deficiency Red cell fragmentation PNH Cold autoimmune haemolysis
Extravascular haemolysis
Haemoglobinopathies
Hereditary spherocytosis
Haemolysis disease of newborn
Warm autoimmune haemolysis
Sickle cell disease: thrombotic crisis precipitant
Infection, dehydration, deoxygenation
Sickle cell disease: thrombotic crisis features
Pain
Sickle cell disease: sequestration crises
Sickling within organs causing pooling of blood with worsening anaemia
Associated with high reticulocyte count
Sickle cell disease: acute chest syndrome
Dyspnoea, chest pain, pulmonary infiltrates, low pO2
Sickle cell disease: aplastic crises
Sudden infection with parvovirus
Sudden fall in Hb
Bone marrow suppression and reduced reticulocyte count
Hogdkins Lymphoma: Staging I
single LN
Hogdkins Lymphoma: Staging II
2+ LN on same side of diaphragm
Hogdkins Lymphoma: Staging III
nodes on both sides of diaphragm
Hogdkins Lymphoma: Staging IV
Beyond LN
Hogdkins Lymphoma: Staging A
No systemic symptoms
Hogdkins Lymphoma: Staging B
B symptoms
Polycythaemia Vera; cause
JAK2 mutations
Polycythaemia Vera; features
Hyperviscosity, pruritus, splenomegaly, haemorrhage, plethoric appearance, low ESR
Polycythaemia Vera; Mx
Aspirin, venesection, chemotherapy
Wiskott-Aldrich Sx; features
Recurrent bacterial infections
Eczema
Thrombocytopenia
Low IgM
What is acute intermittent porphyria?
Rare AD condition
Results in accumulation of breakdown of haem produtcts
Acute intermittent porphyria: features
Abdo pain, vomiting Neuro Psychiatric HTN Tachycardia
Acute intermittent porphyria: Mx
Deep red urine
Raised urinary porphobilinogen
Skin prick test
Most common
Used for food allergies and pollen
RAST
Used for food allergies, pollen and wasp venom
Skin patch testing
Contact dermatitis
Target cells
Sickle cell Thalassaemia IDA Hyposplenism Liver disease
Tear drop
Poikilocytes
Myelofibrosis
Spherocytes
Hereditary spherocytosis
Autoimmune haemolytic anaemia
Basophilic stippling
Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia
Howell-Jolly bodies
Hyposplenism
Heinz Bodies
G6PD deficiency
Alpha thalassaemia
Schistocytes
‘Helmet cells’
Intravascular haemolysis
Mechanical heart valve
DIC
Pencil pikilocytes
IDA
Burr cells
Uraemia
Pyruvate kinase deficiency
SERM e.g.
tamoxifen
SERM: SE
menstrual disturbance
hot flushes
VTE
endometrial Ca
Aromatase inhibitors e.g.
anastrazole
letrozole
Aromastase inhibitors: SE
OP Hot flushes Arthralgia Myalgia Insomnia
Aplastic anaemia: Mx
Blood products Prevention and treatment of infection Anti-thymocyte globulin (ATG) Anti-lymphocyte globulin (ALG) Stem cell transplant
CLL: features
Lymphocytosis Anorexia Weight loss Bleeding, infections LN
CLL: blood film
smudge cells
CLL: Ix
immunophenotyping
Hereditary spherocytosis: features
failure to thrive
jaundic, gallstones
Splenomegaly
Aplastic crisis
Hereditary sphereocytosis: Ix
EMA binding test
Leukaemoid reaction: causes
Severe infection / haemolysis
Massive haemorrhage
Metastatic cancer
Leaukaemoid reaction: features
High leucocyte alkaline phosphatase score
Dohle bodies in WC
Left shift of neutrophils
What is sideroblastic anaemia?
Deposition of iron in mitochrondira due to failure of RC incompletely forming haem
Sideroblastic anaemia: Blood film
Basophilic striping of RBCs
Sideroblastic anaemia: bone marrow
Prussian blue staining
Thymoma associations
Myasthenia gravis red cell aplasia dermatomyositis SLE SIADH
CA 15-3
Breast cancer
S-100
Melanoma
Schwannonmas
Bombesin
Small cell LC
Gastric Ca
Neuroblastoma
What is warm haemolytic anaemia?
Haemolysis at body temperature
Tends to be extra vascular
What is cold haemolytic anaemia?
Haemolysis at 4oC and is complement mediated
Warm haemolytic anaemia e.g.
SLE
Lymphoma, CLL
Methyldopa
Cold haemolytic anaemia e.g.
Lymphoma
Mycoplasma
EBV
t(9;22)
Philadelphia chromosome
CML
t(15;17)
Acute promyelocytic leukaemia
t(8;14)
Burkitt’s lymphoma
t(11;14)
Mantle cell lymphoma
t(14;18)
Follicular lymphoma
Hairy cell leukaemia; features
Pancytopenia
Splenomegaly
Vasculitis
TRAP stain positive
Lead poisoning; features
Abdo pain Peripheral neuropathy Fatigue Constipation Blue lines on gum margin
Lead poisoning; FBC
Basophilic stripping and clover leaf
Polycythaemia; causes
Dehydration Stress Rubra vera COPD Altitude OSA
What is factor v leiden?
Resistance to protein C
CLL: Indications for Rx
Progressive marrow failure Massive LN or splenomegaly Progressive lymphocytosis B symptoms Autoimmune cytopaenias
Universal Donor
AB RhD negative
Aflatoxin
HCC
Aninline dyes
TCC
Asbestos
Mesothelioma and bronchial carcinoma
Nitrosamines
Oesophageal and gastric cancer
Vinyl chloride
Hepatic angiosarcoma
AML: features
Anaemia Neutropenia Thrombocytopenia Splenomegaly Bone pain
AML: poor prognostic features
> 60 years
20% blasts after chemo
Deletions of chromosome 5 or 7
Blood products - Non-haemolytic febrile reaction: features
Fever / chills
RBC 1-2%, platelets 10-30%
Blood products - Non-haemolytic febrile reaction: Mx
Stop / slow transfusion
Paracetamol
Monitor
Blood products - Minor allergic reaction: features
Prutitus
Uriticaria
Blood products - Minor allergic reaction: Mx
Temporarily stop
Antihistamine
Monitor
Blood products - acute haemolytic reaction: features
Fever, abdo pain, hypotension
Blood products - acute haemolytic reaction: Mx
Stop transfusion
Confirm diagnosis (identity, send blood back)
Supportive care
Eosinophilia: pulmonary causes
Asthma Allergic bronchopulmonary aspergillosis Churg-Strauss Loffler's Tropical pulmonary eosinophilia Eosinophilic pneumonia Hyperesoinophilic syndrome
Eosinophilia: infective
Schistosomiasis
Nematodes
Cestodes