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WIS

1/8/18
2 years old child is brought to your clinic for the
evaluation of pallor that mother has noticed for
past few months, and she had also observed that
she grinds her teeth during night

• How will you proceed?


– History
– Examination
– Workup
• Height and weight at 5th centiles
• Palmar pallor present
• No hepatosplenomegaly/lymphadenopathy

Hb: 7.8, hct: 24%


Plt 850
MCV: 64
Retic 0.2%
Smear: microcytic hypochromic
AGE OF ONSET:
• Hemolysis and hemorrhage- ABO, Rh incompatibility.
G6PD deficiency or spherocytosis  at birth

• Erythroblastopenia of infancy 4 months

• Nutritional anemia and hemoglobinopathies  6


months to 2 years

• Fanconi’s anemia 4 to 6 years


• Gender:
– G6PD deficiency  Male
– Iron deficiency due to menstrual loss of
bloodAdolescent girls

• Race:
• Fatigue/ tiredness, dyspnea, palpitations, swelling

• Hx of gall stones, recurrent jaundice hemolytic


anemia

• Hx Pica, chronic diarrhea, worms, GI bleed, prior


surgery, acute and prolonged infections, liver and
renal diseases

• Hx tingling sensation, history suggestive of


neuropathy, oral ulcers Vit B 12 deficiency.

• Hx of rash, joint pain, alopecia Autoimmune


• Diarrhea
– Malabsorption of VitB12/E/Fe
– Inflammatory bowel disease anemia of chronic disease
with or without blood loss
– Change of milk Milk protein intolerance induced blood
loss
– Hx of Intestinal resection  Vit B12 def

• Infections
– Giardia : iron malabsorption
– Intestinal bacterial overgrowth: Vit.B12 def
– EBV, CMV, Parvovirus : Marrow suppression
– Mycoplasma, Malaria : Hemolysis
– Hepatitis : Aplastic anemia
– Hx of Travel to/from areas of endemic infection malaria
or tuberculosis
• Past Medical Hx:
– Previous CBC, Hx of transfusions, Hx of Noenatal
Jaundice
• Nutritional History:
– Cows milk consumption  iron def
– Strict vegetarian  Vit B12 def
– Goats milk  Folate def
– Detailed dietary History including calories
consumption/day
• Use of low iron formula
• Introduction of unmodified cow's milk before the
age of one year
• Excessive milk intake (>24 ounces per day)
• Poor intake of iron-rich foods (meats or fortified
infant cereal)
• Drugs:
– Sulfa, primaquine, henna G6PD def
– Chemotherapy/ chloramphenicol Bone marrow
suppression/aplastic anaemia
– Phenytoin/AEDS Folate def
– NSIADs GI blood loss Fe def
– Exposure to paint, home renovations, or use of
imported or glazed ceramics  lead toxicity
Family History
• Family history of anemia, requirement for blood
transfusions and demise of children Thalassemia
major.

• Hx congenital anemias, hemolytic anemias.


– X-linked: G6PD def
– Autosomal dominant: Spherocytosis.
– Autosomal recessive: Sickle cell anemia , Fanconi anemia
– Family member with early age of cholecystectomy/
splenectomy
• Hx of anaemia following drug use G6PD
Examination
• VitalsPulse, blood pressure and respiratory
rate should be recorded.

• Look for puffiness, edema feet, sacral edema,


jugulovenous pulse, heptic tenderness,
hepatojugular reflux and basal crepitations.
• Facies:
– Hemolytic facies with frontal and parietal bossing,
large head, depressed bridge of nose, malar
prominance, irregular maxillary teeth.

– Diamond Blackfan syndrome will have box like face

– Look for periorbital puffiness which can suggest


edema due to anemia, CCF

– Dysmorphism, microcepahly Fanconi


• Eyes:
 Fanconi’s anemia microcornea
 Conjunctival vessels tortuosity sickle cell
anemia
Icterus hemolytic anemia

• • Oral cavity:
Look for glossitis, angular stomatitis, bald tongue
 suggest nutritional anemia.
Look for teeth abnormality for hemolytic anemia.
• HANDS
• Nail changes:
– Koilonychia, brittle nails -- iron deficiency
– Dyskeratotic nails -- dyskeratosis congenita
• Absent thumb/bifid thumb
• Dactylitis/sausage fingers
• Palmar Pallor (Look at creases, evident when
Hb<7g/dl)

• Lymphadenopathy-- tuberculosis, HIV, infectious


mononucleosis,leukemia, lymphoma
• Hepatosplenomegaly:
– Palpable tender liver with positive hepatojugular
reflux -- CCF.

– Hepatosplenomegaly -- tuberculosis, HIV,


leukemia, thalassemia, other hemoglobinopathies,
lymphoma, myelodysplastic syndrome, CML,
malaria, kala azar

– Isolated splenomegaly -- enteric fever, malaria,


portal hypertension, lymphoma, CML, tropical
splenomegaly or hypersplenism, immune
hemolytic anemia, congenital spherocytosis
• Bleeding manifestation:
– Petechiae, purpura – ITP or aplastic anemia, malignancies or marrow
infiltration.

• Skeletal changes:
– Fanconi’s anemia, TAR syndrome-- absent radius, absent or bifid
thumb, triphalangeal thumb, polydactyly, syndactyly, short
stature,microcephaly

– Look for associated anomalies like mental retardation, skin


hyperpigmentation, hypogonadism, renal anomalies in such cases.

• Skin changes:
– Hyperpigmentation -- Fanconi’s anemia
– Icterus -- liver diseases, hemolytic anemia
– Non-healing ulcers over lower limbs -- chronic hemolytic anemia
– Localized DIC like picture with anemia and thrombocytopenia --giant
cavernous hemangioma  Kasabach-Merrit syndrome.
Thank You

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