Haematology Flashcards
Normal Hb levels
Male: 130 - 180 g/L|13.8 to 17.2
Female: 115 - 165 g/L | 12.1 to 15.1
Normal serum ferritin level
men: 24 -336 mg/L
women: 11 - 307 mg/L
Mean corpuscular count
80 - 100 fL
Platelet count
150 - 400 x 10^9/L
Bleeding time
2 - 8.5 mins
Prothrombin time (pt)
10 - 13 secs
Partial thromboplastin time (aPTT)
25 - 35 secs
Autosomal dominant
- spherocytosis
- hemorrhagic telangiectasia
- Von Willebrand disease
Autosomal recessive
- Factor 5 diseases
- Fanconi’s anaemia
- haemochromatosis
X-linked recessive
Haemophilia A
Haemophilia B
- Glucose-6-phosphate dehydrogenase deficiency
rise in platelet count causes
– Polycythemia vera.
– Hyposplenism due to splenectomy.
– Iron deficiency anemia.
– Correction of vitamin B12 and folic acid deficiency
microcytic anemia + . Seizures
+ peripheral neuritis + dermatitis
Vitamin B6 (pyridoxine) deficiency
vegan vegetarian deficiency
B12 deficiency
Vitamin B12 bsorbed by binding to
intrinsic factor
B12 deficiency causes what type of anaemia
pernicious anaemia
B12 deficiency conditions
- Autoimmune gastritis
- Terminal ileum disease
- Gastrectomy
- Metformin therapy
- Coeliac disease
- H.pyelori infection
- Crohn disease
- Postgastrectomy
weakness + fatigue + dizziness + palpitations + exercise intolerance + craving of ice/clay
Iron deficiency
increased MCV + neutropenia + thrombocytopenia + history of alcohol abuse + hypersegmentation of
the neutrophils
Folate deficiency
cheilosis + glossitis+ a variety of ocular problems + hx of biliary atresia/hepatitis
Folate deficiency
px around 70 + fatigue + tiredness + macrocytic anaemia
myelodysplasia
myelodysplasia investigation
Bone marrow biopsy
Causes of macrocytic anemia
High MCV
megaloblastic anemias
- Vitamin B12 (cobalamin )
- folate deficiency
- myelodysplasia
vigorous reticulocytosis,
hypothyroidism,
chronic liver disease,
myelodysplastic syndrome
Common causes of microcytic anemia
Low MCV
iron deficiency
thalassemia,
anemia of chronic disease
sideroblastic anemia
difference between hemolytic anemia vs anemia of chronic disease
Low hepatoglobin
congenital developmental anomalies + progressive pancytopenia
Fanconi’s anaemia
Fanconi anaemia investigation
Diagnostic: Chromosome fragility test
Fanconi anaemia congenital abnormalities
- Hand and arm anomalies (misshapen,missing or extra thumbs or abnormalities of
the radius) - Skeletal anomalies of the hips, spine or ribs
- Skin discolouration (café au lait spots, hyper-pigmentation,
- Small head or eyes
- Low birth weight and subsequent short stature
- Missing or horseshoe kidney
- Gastrointestinal abnormalities including abnormal development of the
oesophagus
sudden onset of jaundice + pallor, +
dark urine + with or without abdominal and back pain + fall in the haemoglobin concentration + “bite” cells
Glucose-6-phosphate dehydrogenase deficiency
acute haemolysis following ingestion of cotrimoxazole/primaquine
Glucose-6-phosphate dehydrogenase deficiency
Glucose-6-phosphate dehydrogenase deficiency dx
Heinz Bodies
helmet cells + Artificial (mechanical) valves placemenent
schistocytes Hemolytic anemia
schistocytes Hemolytic anemia lab findings
Decreased serum haptoglobin level
spherocytosis clinical features
1-Anaemia and jaundice.
2-Splenomegaly.
3-Positive family history of anaemia, jaundice, or gallstones.
4-Spherocytosis with increased reticulocytes.
5-Increased osmotic fragility.
6-Negative direct antiglobulin test (DAT).
The outcome of splenectomy in a patient with spherocytosis
1- Spherocytosis persists after splenectomy, the cells survive longer in the circulation.
2-Decrease in reticulocytosis.
3- RBC fragility remains high resulting short life span of red blood cells
4- Fall in elevated bilirubin
5- In severely affected patients, life-threatening anaemia
Spherocytosis gene defect
spectrin
ancyrin
protein 4.1
fever + thrombocytopenia + low haemoglobin + acute renal failure + hallucination + haemolytic anaemia
Thrombotic thrombocytopenic purpura (TTP)
immediate hemolytic reaction
secondary to a blood transfusion invesitgation
Positive Coombs test
Thrombotic thrombocytopenic purpura (TTP) treatment
- replacement fluid
- fresh frozen plasma
pruritis on hot bath + vertigo + tinnitus + headache + visual disturbances + hypertension + transient ischemic
strokes
Polycythaemia vera
fever + maculopapular rash+ serum AST, bilirubin and alkaline phosphatase elevation post transplant
Graft-versus-host disease
decreased serum albumin + hypercalcaemia + anaemia + bony lytic lesions/osteoporosis
Multiple myeloma
CRAB
C - hypercalcaemia 13%
R - renal impairment 20–40%
A - anaemia 70%
B - bony lesions
Multiple myeloma investigation
Initial: & diagnostic: serum and urinary
electrophoresis
poor prognosis of multiple myeloma
- higher levels of beta-2 microglobulin
- lower levels of albumin
hepatomegaly + weakness+ hyperpigmentation + atypical
arthritis + diabetes + impotence + unexplained chronic abdominal pain +
cardiomyopathy + Decreased production of FSH and LH + gynaecomastia + 90% are C282Y homozygotes
haemochromatosis
Most common manifestation of cardiac
involvement in hemochromatosis
Congestive cardiac failure
most common cause of death in hemochromatosis
- Liver disease
- cardiomypathy
haemochromatosis investigation
Serum transferrin saturation
- >60% men
- 50$ women
haemophilia A in pregnancy
- measure Factor 8 level at 1st antenatal
visit and at 32 weeks - majority develop normal level of factor 8 and do not require replacement
-Malaise and pallor + Recurrent infection + Gingival hypertrophy + Fever, night sweats + -Normocytic normochromic anaemia + Thrombocytopenia + myeloblasts more than 20%
acute myeloid leukaemia (AML)
conditions which can lead to the development of AML
-Trisomy 21 noted in Down syndrome.
-Fanconi anaemia.
-Ataxia-telangiectasia.
-Myeloproliferative syndromes.
pallor + sweats + weight loss + bilateral axillary lymphadenopathy + massive Spleen and liver megaly
Chronic lymphocytic leukaemia (CLL)
CLL management
1st line: corticosteroids such as prednisolone
chemotherapy if needed
urticaria + stridor + hypotension during blood transfusion+ hx of recurrent infections
Immunoglobulin A deficiency
fatigue and weakness, dyspnea, and pallor + dysplasia + Anaemia with/o f bi- or
pancytopenia+ Macrocytosis + absolute neutropenia
Myelodysplasia
common feature of autoimmune thrombocytopenia in
childhood
Antecedent viral illness
microcytic anemia + increased concentration of hemoglobin A2
β-Thalassemia
β-Thalassemia investigation
diagnostic: hemoglobin electrophoresis
most profound adverse effect of chronic
transfusional iron overload in children with thalassemia?
Progressive hepatic cirrhosis
sickle cell anaemia reduce iron overload
- Subcutaneous deferoxamine
- Splenectomy
- ## Erythrocytopheresis
earliest complication of sickle cell disease
Bone infarction
decreased red cell, white cell and platelets counts
pancytopenia/aplastic anemia
aplastic anaemia causes
NSAIDs
- Sulfonamides.
– Gold.
– Anti-epileptic drugs (carbamazepine, valproic acid, phenytoin).
– Nifedipine.
– Chloramphenicol.
generalised weakness, low-grade fever and sore throat + normal haemoglobin + low neutrophil + low platelets + thyroid medicatiob
agranulocytosis
Long-standing history of prolonged bleeding after trauma and history of menorrhagia in an otherwise healthy woman
Von Willebrand disease
fever, night sweats, weight loss + painless generalized lymphadenopathy which becomes painful after alcohol consumption
f Hodgkin lymphoma (HL)
f Hodgkin lymphoma (HL) highest risk of malignanc
Supraclavicular lyphadenopathy
Hodgkin lymphoma (HL) diagnostic investigation
Lymph node excision biopsy
non-Hodgkin lymphoma features
GI tract most common site
- 3% of all malignant gastric tumors
- Surgery alone can be considered adequate treatment that does not infiltrate beyond the submucosa
-
incidence of thrombosis and bleeding in patients with myeloproliferative neoplasms?
- typically mucocutaneous
- Bleeding is usually less severe and less frequent than thrombosis
- Arterial
thromboses are more common than venous thrombosis - Strokes are more frequent followed by myocardial infarction and peripheral arterialocclusion
best test to monitor heparin therapy
Activated partial thromboplastin time (aPTT)
DVT in the presence of thrombocytopenia
heparin induced thrombocytopenia
types of heparin induced thrombocytopenia
Type 1 : first 2 days after exposure, platelet count normalizes with continued heparin therapy. non-immune
Type 2: typically occurs 4-10 days after
exposure, immune-mediated disorder
- Venous limb gangrene
- Bilateral adrenal hemorrhagic infarction
- Skin lesions at injection sites
- Acute systemic reactions following an i- ntravenous heparin bolus
heparin induced thrombocytopenia management
discontinue and avoid all heparin products immediately
Renal failure reflected by oliguria and abdominal pain following invasive diarrhoea
hemolytic uremic syndrome (HUS).
