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Haematology Flashcards

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1
Q

What is the management for sickle cell disease?

A
  1. Early aggressive treatment for infections with prophylactic antibiotics and appropriate vaccinations
  2. Folate supplement
  3. Hydroxyurea: to increase HbF concentration
  4. Long-term follow-up
    >> Transcranial doppler: stroke risk assessement
    >> Starting at 10 years of age: Annual fundoscopy
    >> Biannual screening for pulmonary hypertension
    >> Biannual urinalysis
  5. Chronic transfusion
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2
Q

What are the 3 underlying pathophysiological causes of physiological anemia of infancy?

A
  1. HbF has 2/3rd of the half-life of HbA
  2. Increased oxygen outside the uterus and the increased oxygen affinity of HbF render less RBCs/Hb necessary after birth
  3. Rapid expansion of plasma volume in a newborn

>> Hb 18g/dL at birth of a term infant
>> Hb 8-11g/dL at 2 months of a term infant
>> Reticulocyte count at birth: 5%
Reticulocyte count at 1 week of life: 1%

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3
Q

What are the complications of Hemophilia A?

A
  • Anti-FVIII antibodies: “inhibitors” – present in 5-20% of patients
  • Transfusion-related complications
    >> Line infection
    >> Symptoms of iron overload
    :: Liver cirrhosis
    :: Diabetes (pancreatic deposition)
    :: Cardiomyopathy
    :: Arthritis
    :: Testicular failure
    :: Slate grey discoloration of the skin
  • Problems with vascular access for transfusion
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4
Q

What is the treatment for B-thalassemia major?

A
  • Life-long regular transfusion with Fe chelation +/- folate supplements
    >> Signs of iron overload
    :: Liver cirrhosis
    :: Cardiac myopathy
    :: Diabetes mellitus from pancreatic deposition
    :: Infertility (testicular failure) and delayed growth from pituitary deposition
    :: Hyperpigmentation of the skin
    :: Arthritis
    >> Chelating agents
    :: Deferoxamine (SC infusion)
    :: Deferasirox (PO)
    :: Deferiprone
  • Hydroxyurea (in thalassemia intermedia): to increase HbF levels
  • Splenectomy
  • Bone Marrow Transplant
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5
Q

What are the long-term complications of sickle cell disease?

A
  • Growth delay
  • Bone abnormalities, eg. AVN
  • Gallstones
  • Retinopathy
  • Restrictive lung diseases
  • Cardiomyopathy with pulmonary hypertension
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6
Q

What can DDAVP be used to treat?

A
  • Central diabetes insipidus
  • Nocturnal enuresis
  • *- Hemophilia A
  • vWF disease**
  • Uremic platelet dysfunction

>> DDAVP helps release vWF and thus factor VIII

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7
Q

What are the reference numbers of anemia in childhood?

A

Newborn: ~18g/dL
3 months: ~8-10g/dL
Adult female: ~12-16g/dL
Adule male: ~14-18g/dL

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8
Q

What are the CBC reference ranges for neonates?

A

Hb: 14-21g/dL
WBC: 10-25 x 10^9/L
PLT: 150-400 x 10^9/L

Therefore, Hb and WBC levels are elevated in normal neonates, while the PLT levels should be similar to those of normal adults.

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9
Q

What is the typical age at presentation of iron deficiency?

A

6 months - 3 years

>> Iron stores last ~6 months in term infants
>> Iron stores last ~2-3 months in preterm infants

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10
Q

What is usually the first sign of vaso-occlusive crisis in sickle cell disease?

A

Dactylitis

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11
Q

What are the features of lead poisoning?

A

L: Lead lines on gingivae and epiphyses of big long bones on X-ray
E: Encephalopathy; Erythrocyte basophilic stippling
A: Anemia (microcytic); Abdominal colic
D: Drops of the wrist and foot

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12
Q

What is the Mentzer index?

A

= MCV/RBC count

  • Thalassemia: <13
  • Iron-deficiency anemia: >13
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13
Q

What are the three types of crises in sickle cell disease?

A
  • Vaso-occlusive crisis
  • Aplastic crisis
  • Acute splenic sequestration
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14
Q

What are the possible causes of bleeding tendencies in children?

A

Platelet problem
- Quantitative
>> Increased consumption
:: DIC
:: Giant hemangioma
:: Hypersplenism
>> Increased destruction
:: ITP
:: TTP/HUS
:: HIT
>> Decreased production
:: Aplastic anemia
:: Marrow infiltration
:: Leukemia/lymphoma
:: Drugs
- Qualitative
>> Congenital
:: Bernard-Soulier
:: Glanzmann’s thromboasthenia
>> Acquired
:: Drugs (ASA, NSAIDs, alcohol)
:: Uremia

von Willebrand disease

Coagulation pathway problem
- Congenital
>> Hemophila A
>> Hemophilia B
- Acquired
>> Vit K deficiency – hemorrhagic disease of newborn
>> Liver disease
>> DIC Vascular problem
- Congenital
>> Connective tissue disorders
:: Ehlers-Danlos syndrome
:: Marfan syndrome
>> Osler-Weber-Rendu syndrome
- Acquired
>> Henoch-Schonlein Purpura
>> Cushing’s syndrome
>> Infections
>> Drugs
>> Purpura simplex

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15
Q

What are the drugs to avoid in a G6PD patient?

A

Hemolysis IS PAIN.

I: Isoniazid
S: Sulfonamides
P: Primaquine
A: Aspirin
I: Ibuprofen
N: Nitrofurantoin

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16
Q

What is the peak age of onset for immune thrombocytopnic purpura (ITP)?

A

2-6 years (2-10 years)

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17
Q

What is the most common cause of thrombocytopenia in childhood?

A

Immune thrombocytopenic purpura

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18
Q

What are the presenting features of ITP?

A

Usually 1-3 weeks after viral illnesses (URTI, chickenpox)

  • Sudden onset of petechiae, purpura and epistaxis
  • Otherwise well
  • No lymphadenopathy
  • No hepatosplenomegaly
  • *CBC**
  • PLT low
  • Hb normal
  • WBC normal
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19
Q

What are the possible complications of ITP?

Study These Flashcards
A
  • GI bleeding
  • Intracranial hemorrhage (<10 PLT) — Chance: <0.5%
20
Q

What are the indications for a bone marrow aspirate in ITP?

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A
  • >1 cell line abnormal
  • Hepatosplenomegaly present
  • Before steroid treatment

>> Steroid treatment can mask the features of ALL and thus further delay diagnosis and treatment

21
Q

What is the management for ITP?

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A
  1. Conservative
    • Spontaneous recovery in >70% within 3 months
    • Avoid contact sports and NSAIDs
  2. Medical: usually gum bleeding/PLT <20
    • IVIG
    • IV prednisone
    • Anti-D antibodies
  3. Surgical: for life-threatening bleeding
    • Additional platelet transfusion
    • Emergency splenectomy
22
Q

What is the definition of chronic ITP?

Study These Flashcards
A

PLT remains low 6 months after diagnosis

Management for persistent bleeding affecting daily life:

  • Rituximab
  • TPO factors
  • Screening for SLE
  • Splenectomy
23
Q

How do we classify hemophilia severity?

Study These Flashcards
A

Mild: 5-40% of normal factor VIII levels

Moderate: 1-5% of normal factor VIII levels

Severe: <1% of normal factor VIII levels

24
Q

What is the mode of inheritance of hemophilia A/B?

Study These Flashcards
A

X-linked recessive

25
What is the management of hemophilia A?
**_Active bleeding_** - Recombinant FVIII concentrate \>\> Aim for **30% in minor** bleeds \>\> Aim for **100% for major** OT or life-threatening bleeds :: Maintain at _30-50% for 2 weeks_ :: Regular infusion of factor concentrate (8-12 hourly) * *_Prophylaxis for severe hemophilia_** - Aim for baseline **\>2%** - Regular recombinant factor concentrate infusion - **2-3 times per week, starting at 2-3 years of age** * *_Mild hemophilia_** - **DDAVP**: promotes release of VIII-Ag
26
How can we manage inhibitors to FVIII concentrates in Hemophila A patients?
- Very high doses of FVIII recombinant concentrates - Activated FVII recombinant concentrate
27
What are the causes of anemia in children?
**_Decreased production_** - Red cell aplasia \>\> Congenital :: Aplastic anemia :: Diamond-Blackfan syndrome :: Fanconi's anemia \>\> Acquired :: Parvovirus B19 infection :: Acquired aplastic aenmia :: Leukemia :: Transient erythroblastosis of childhood - Ineffective erythropoiesis \>\> Iron deficiency \>\> B9/B12 deficiency \>\> Chronic inflammation: JIA, IBD \>\> Chronic renal failure \>\> Myelodysplasia \>\> Lead poisoning * *_Increased destruction_** - RBC enzyme defect: G6PD deficiency - RBC membrane defect: hereditary spherocytosis/elliptocytosis - Hemoglobulinopathy: thalassemia, sickle-cell disease **_Blood loss_** - Fetomaternal bleeding - Chronic GI blood loss - Inherited coagulopathies \>\> vWF disease \>\> PLT dysfunction \>\> Coagulation dysfunction \>\> Vasculitis
28
How does one approach anemia in children?
**_By MORPHOLOGY_** ## Footnote * *_Microcytic_** - **Iron deficiency** - **Thalassemia** - Anemia of chronic disease - Sideroblastic anemia - Lead poisoning **_Normocytic_** - High reticulocyte count (destruction problem) \>\> Bleeding \>\> Hemolysis :: Enzyme defect: G6PD deficiency :: Membrane defect: spherocytosis :: Other hemogloblinopathies: sickle cell :: Immune causes ~ Drugs ~ Infection: B19, malaria :: **Microangiopathic anemia ~ DIC ~ HUS/TTP** - Low reticulocyte count (production problem) \>\> Pancytopenia :: Aplastic anemia :: Myelodysplastic syndromes :: Leukemia :: Amyloidosis/sarcoidosis :: **TB** :: Bone marrow-suppressive drugs: chemotherapy \>\> Isolated anemia :: Anemia of chronic disease :: Renal disease (decreased EPO production) :: Liver disease **_Macrocytic_** - Megaloblastic \>\> B12 deficiency \>\> Folate deficiency \>\> Drug-induced :: Methotrexate :: Sulfonamides :: Chemotherapy - Non-megaloblastic \>\> Liver disease \>\> **Hypothyroidism** \>\> Reticulocytosis
29
What is the chronological order of response to iron supplement in iron-deficiency anemia?
- Increased reticulocyte count in 2-3 days - Increased hemoglobin levels in 4-30 days - Iron store repletion in 1-3 months
30
What are the complications of iron deficiency?
- Developmental delay if untreated - Angular cheilitis - Glossitis - Koilonychia
31
Which inflammatory mediator is involved in anemia of chronic disease?
**IL-6** with **increased hepcidin**
32
What are the causes of sideroblastic anemia?
* *_Congenital_** - X-linked hereditary - Treat with **high-dose vitamin B6** **_Acquired_** - Idiopathic: preleukemic phenomenon (10% to AML) - Reversible ** \>\> Drugs :: Isoniazid :: Chloramphenicol** \>\> Alcohol \>\> **Lead** \>\> Zinc \>\> **Hypothryoidism**
33
What are the causes of basophilic stippling in RBCs on a peripheral blood smear?
**TAIL** ## Footnote T: Thalassemia A: Anemia of chronic disease I: Iron deficiency L: Lead poisoning
34
What are the causes for target cells on a peripheral blood smear?
**HALT** ## Footnote H: HbC disease, sickle cell disease A: Asplenia L: Liver disease T: Thalassemia
35
What are the causes for aplastic anemia in children?
Congenital - Fanconi's anemia - Schwachman-Diamond syndrome Acquired - Idiopathic - Post-viral infection \>\> Parvovirus B19 \>\> EBV \>\> HBV, HDV, HEV \>\> HHV-6 \>\> HIV - Drugs \>\> Chloramphenicol \>\> Phenylbutazone \>\> Chemotherapy - Toxins - Ionizing radiation - Autoimmune \>\> SLE \>\> Graft-versus-host disease
36
What are the laboratory findings in hemolytic anemia?
- **Increased reticulocyte count** - **Decreased haptoglobin** - Increased _unconjugated_ bilirubin - Increased LDH - _Increased urobilinogen_
37
What are the clinical features of B-thalassemia major?
**Onset at 6-12 months \>\> HbF to HbA at 3-6 months** ## Footnote - Severe anemia and jaundice - Maxillary overgrowth - Frontal bossing - Gross hepatosplenomegaly - Skull X-ray: hair-on-end/crew-cut appearance - Pathological fractures common - Pigmented gallstones
38
What is the mode of inheritance of thalassemia?
Autosomal recessive
39
What are the complications of iron overload?
- Cardiac failure - Liver failure/cirrhosis - Diabetes - Infertility - Growth failure - Arthritis - Skin pigmentation
40
How many b-chain genes do we have?
2 on chromosome 11
41
How many a-chain genes do we have?
4 on chromosome 16
42
What are the different types of a-thalassemia?
1 defective gene: clinically silent (trait) 2 defective genes: normal Hb, decreased MCV (trait) 3 defective genes: HbH (b4) disease 4 defective genes: Hb Barts (gamma4) disease - hydrops fetalis; incompatible with life
43
What is a specific investigation for vW disease?
**Ristocetin-induced platelet aggregation test:** decreased in von Willebrand disease
44
What is the mode of inheritance of von Willebrand disease?
Mostly autosomal dominant
45
What is the management for von Willebrand disease?
* *- Desmopressin - Tranexamic acid** - High-purity FVIII concentrate - Conjugated estrogens to increase vWF levels
46
What are the common presenting features of hemophilia A?
**_Five Hs_** ## Footnote - Head hemorrhage - Hematochezia - Hematuria - Hemarthosis - Hematomas
47
What are the types of vW disease?
Type I: mild quantitative disease (~75%) Type II: qualitative disease (~20-25%) Type III: severe quantitative disease (rare)

Paediatrics (16 decks)

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