Haematology / Oncology Flashcards
Myeloma - ft / Inx
Neoplasm of BM plasma cells –> 60 - 70 yrs
Ft:
- Bone - pain, osteoporosis, pathological #. Osteolytic lesions
- Lethargy
- infection
- HYPERCA
- Renal Fx
- other ft: amyloidosis.
Ins:
- IgG or IgA !!!!
- Urine –> BENCE - JONES protein
- whole body MRI
- Skull xray –> RAIN DROP SKULL
Hyper Ca of myeloma:
- Primarily due to increased osteoblastic resorption due to local cytokines (IL-1/TNF) = released by myeloma cells:
- other causes = impaired renal fn/increased renal abs of Ca/ elevated PTH-rP
Myeloma diagnostic criteria
Major:
- > 30% plasma cells in BM
- Plasmacytoma - BM sample
- Elevate M protein
Minor:
- 10% - 30% plasma cells –> BM sample
- Minor elecation of M protein
- Osteolytic lesions
- low levels of Ab
Myeloma prognostic indicator?
B2 - macroglobulin
increased macroglobulin is worse survival.
MGUS
Ft:
- Asx
- no bone pain or inc risk of infection
- Demyelinating neuropathy
MGUS vs Myeloma:
- No beta-2 macroglobulin
- normal immune fn
- lowere paraporteinaemia (igG and IgA)
- Paraproteinaemia doesn’t increase.
- no lytic lesions or renal dx
Complications of. CHOP regimen
Used in NHL
Can get neutropaenic sepsis
High risk of tunour lysis - - “> give allopurinol
Causes of macrocytic anaemia
Low Vit B12:
- found in red meat/fish
0absorbed terminal ileum
- Bound to IF
- Pernicious anemia - Ab vs GAstric parietal cells, therefore get low IF –> do schillin test which is measure uriniary B12 w/ and w/o IF.
- No neuro signs –> IM HYDROXYCABAAMIN
- If foloic A deficient as well –> replace folic A first - avoid SCDC
Folic A:
- found in FOLIAGE and Liver
- absorbed in Jejenum
Drugs that affect nuceleic Acid synthesis:
- MTX
- Hydroxycarbamide
- Aza
Macrocytosis w/ normoblasic BM
Alcohol -->low folate Liver dx --> TARGET CELLS Reticulocytosis -- RETICULOCYTES - acute blood loss or HA - PRegnancy - hypothyroid - myelodysplasia -- > Cytopenias. BM = DYSPLASTIC - myeloprolif --> TEAR DROP i BM Myeloma
Microcytic anaemia
IDA:
- Pencil cells
- hypochromic
- Fe. TIBC. Ferritin
Thalassaemia:
- Meditarannean/asian patient
- beta thalassaemia minor - microcytosis = disproprotionate to anaemia
Anaemia of CD
Sideroblastic
Pb poisoning
Patient with previously normal Hb –> acute px of low MCV and not at risk of thalassaemia —- what is it?
polycythaemia vera
IDA secodary to bleeding
Thalassaemias
Alpha thalamssaemia:
- Chromsome 16
- required for HbA/HbA2/HBF
- Severity dependent # of allles affected:
- 1 or 2 allele –> Normal Hb
- 3 alleles –> low Hb and MCV
- > 4 alleles –> in utero death = hydrops fetalis/barts hydrops
Beta thal major:
- Chromosome 11
- Absence of bet chain production
- px = 1st yr of life with FX TO THRIVE + HEPATOSPLENOMEGALY
- ow MCV
- HbF/HbA2 = raised
- HbA = Normal
- Tx = RPT TRANSFUSIONS (Fe overload) / SC DESFERRIOXAMINE
Beta - Thal Trait:
- HbA2 - raised
- Hb minor drop - remains >90
- ASx
Sickle cell anaemia
AR - more common in african decent as provides protection vs Malaria
Polar glutamate is switched for non-polar valine –> increased susceptible to hypoxia –> Polymeruse –> Sickling
Sickle RBC –> HAemolyse –> block BV –> Infarction
HbAS = hetero = sickling at pO2 of 2 --> 4.5 HbSS = homo = sickling at pO2 of 5 --> 6
Inx: Hb electrophoresis
Mx:
- Hydroxyurea - inc HbF = PROPHYLAXIS vs sickle crisis
- pneumococcal vax
Sickle cell Crises
Thrombotic:
- painful vaso-occlusive crisis
- precipitants = low O2/ infeection/ dehydration
- infarction of bones/organs - AVN/gut/lungs.brain
Sequestration:
- Pooling of blood in lungs + spleen
- WORSENING ANAEMIA
Acute chest sydndrome:
- CHEST PAIN + SOB + LOW SATS + PULM INFILTRATES:
- most common cause of death in adults.
Aplastic:
- Follows infection with PARVOVIRUS
- Sudden drop in Hb
HAemolytic crisis:
- Rare
- Fall in Hb due to haemoysis
Mx: IVI O2 Analhesia consider Abx Bloods transfusion if NEURO SIGNS ---> EXCHANGE TRAINSFUSION!!!!!
Aplastic anaemia
Causes:
- IDiopathic
Drugs - Gold/phhenylbutazone/chloramohenme
- post hepatitis supervening post infection
- Chemo/RT –> Low TPMT when starting Aza/matacopurine
MX:
- blood productions + infection prevention
- Anti0-thymocyte globulin and anti - ymphocyte globulin
- stem cell transplant
Tumour lysis syndrome
Triggered by chemo
BReak down of tumour cells
URic acid hgh
K+ high
PO4- High
Ca Low
IF high risk:
- IV allopurinol or IV rasburicase immed prior to Chemo 1st dose
If lower risk:
- PO Allopurinol
Waldenstroms macroglobulinaemia
Older en
Anaemia monoconcal IgM production Hyperviscosity syndrome hepatosplenomegaly LN
TTP - PENTAD
1) Fever
2) NEuro signs
3) Thrombocytopaenia
4) Haemolysis
5) Renal Fx
HEreditaru angioedema
Think in a young patient with signs of andioedema but not anaphylactoid
AD - Low C1 inhibitor levels
Mx:
- IV C1 inhibitor or FFP if not avail
- prophylaxis - anabolic steroid = Danzol
Fe Metabolism
icnreased by
- Vitamin C
- Gastric H+
Decreased by : PPI Tetracyclin Gastric anchlorydia Tannin
Sideroblastic anaeia
RBC fx to completely form haem
therefore get Fe depositis in mitochndria –> Sideroblasts
Can be congenital or acquired
Acquired:
- Myelodysplasia - pimary aquired
- AtOH, Anti-TB or LEad = secodnry acquired
Inx:
- Low MCV/MCH
- BM –> Sideroblasts + high Fe Stores
Mx:
- Supportive + tx underlying +/- Pyridoxine
Myeloma - prognosis marker
B2-microglobulin
Haemolytic anaemias by site - intravasc.
Mismatched blood transfusion:
- chest pain, fever , agitation, low BP
- HBO mismatchin –> within misn
- Mx: STOP TRANFUSION + IVI
G6PD:
- Low glutathione –> susceptible to Oxidative stress
- HEINZ BODIES
- neonatal jaundice
- precipitants: SPESIS/BROAD BEANS/CIPRO/PRIMAQUINE
- Inx - G6PD activity
Red cell frgamentation:
- HEart Valves
- TTP/DIC/HUS
Paroxysmal nocturnal haemoglobinuria:
- increase sensitivity to complement
- CD 59 and CD55 activity low
- get haemoglobinuria in early am DARK URINE IN A.M.
Cold AIHA:
- Raynauds
- Acrocyanosis
- IgM!!!!!!
- Causes: lymphoma/mycoplasma/EBV
Haemolytic anaemias - intravasc
TTP / DIC / HUS
TTP:
- Pentad =
- Causes
- Mx
H.U.S: - - Triad: - Secondary to - Blood film --> Mx:
Haemolytic anaemias - intravasc
TTP / DIC / HUS
TTP:
- ADAMST2
- Pentad = FEVER + NEURO + PLT LOW + LOW Hb + RENAL FX
- Causes - GU/GI Infection/ PReg/Ciclosporin + Penicillin + OCP/tumour / SLE/ HIV
- Mx: PLASMA EXHNAGE
H.U.S: - YOUG cchild - Triad: Microangiopathic haemolytic anaemia + low platelets + AKI - Secondary to E.COLI - Blood film --> FRAGMENTED RBC Mx: - Supportive - if Sev w/o diarrhoea --> Plasma exhange
DIC:
- Stress –> TF release –> aExtrinsic + intrinsic pathways.
- Inc PT/APTT/bleeding time
- low plt
- Schistocytes
- FIBRIN DEGRADATION PRODCCTS
- Mx = supportive.
Multiple myeloma mx
Watchful wait
AMjor criteria:
- Plasmacytoma
- 30% plasma cells in BM samle
- Elevated M protein
Haematological malignancies: infections
Virus:
- EBV –> Hodgkins/burkitts/naspharyngeal Ca
- HTLV-1 - Adult T cell leukaemia/lymhoma
- HIV-1 - high grade B-cell lymohoma
BActeria:
- H.pylori - gastric MALT
Protozoa:
- Malaria –> Burkitts lymohoma
Waldenstroms macroglobulinaemia
IgM Paraproteinaemia !!!
MONOCLONAL
Most likely get –> HYPERVISCOSITY SYNDROME
AML
most common leaukaemia - ADULTS
Can be primary or secondary transformation of myeloprolif.
Px –> BM Fx
Poor prognostic:
- > 60 yrs
- > 20% blasts
- Detection of Chromosome 5 or 7
Classification = French - american - british (FAB)
ALL
most common leukaemia in CHILDREN
PRolif of B + T cells
Poor prognostics:
- MAle
- non-caucasian
- <2yr or >10 yrs
- CNS involvement
- philidelphaia translocation
- initial high WCC
- B + T cell surface marker
- FAB L3
Acute promyelocytic leukaemia M3
Assox with t(15,17)
Younger onset
Fusion of PMR + RAR
AUER RODS
DIC + Thrombocytopaenia
CML
BCR - ABL = philideplphia chromosome + 9 and 22
BCR- ABL –> increase in TK ACTIVITY
Ft:
- Splenomegaly –> ABDO PAIN
- granulocyte formatio
MX:
- imatinib –> inhibit TK
CML
BCR - ABL = philideplphia chromosome + 9 and 22
BCR- ABL –> increase in TK ACTIVITY
Ft:
- Splenomegaly –> ABDO PAIN
- granulocyte formatio
- LOW LEUKOCYTE ALANINE
MX:
- imatinib –> inhibit TK
