3rd June 2004 Asymptomatic Jaundice

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3rd June 2004Asymptomatic Jaundice
Dept of Medicine Pathology CWM Hospital Fiji
School of MedicineWeekly CPC Meeting

• Dr. Venkatesh Murthy Shashidhar
• Associate Professor of Pathology

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Patient details

• 17 year old Fiji Indian male
• Chronic jaundice, no other symptoms.
• On examination spleen 1 palpable.
• Laboratory Bilirubin 60-70mg/dl, predominantly
  unconjugated.
• Full blood count results normal.
• High reticulocytes 20 and repeat 22
• Hb 12.0gm.

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Patient details

• Liver function tests within normal limits
  except high bilirubin.
• Provisional diagnosis of chronic hemolytic anemia
  ? Cause.
• Blood film showed plenty of spherocytes, moderate
  polychromasia and occassional nucleated RBc.
• Hereditary Spherocytosis.

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Blood film morphology
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Blood film morphology
Spherocytes. Normal
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Blood film morphology
Spherocytes. Normal RBC
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Blood film morphology
Nucleated RBC
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Blood film morphology
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Discussion Hereditary Spherocytosis

• chronic familial icterus / chronic acholuric
  jaundice / familial spherocytosis
• a chronic disease, inherited as a dominant trait.
• Hemolysis of spheroidal RBCs, anemia, jaundice,
  and splenomegaly.
• Although usually one or more family members have
  had jaundice, anemia, or splenomegaly, one or
  more generations may be skipped because of
  variations in the degree of gene penetrance.

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Discussion

• Mild Symptoms and signs with compensated anemia.
• Moderate jaundice and anemia severe cases.
• Aplastic crises may result from intercurrent
  infection may exacerbate the anemia.
• Splenomegaly is almost invariable but rarely
  causes abdominal discomfort. Hepatomegaly may be
  present.
• Cholelithiasis (pigment stones) is common and may
  be the cardinal symptom at presentation.
• Congenital skeletal abnormalities (eg,
  tower-shaped skull, polydactylism) occasionally
  occur.

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Discussion

• Several RBC membrane protein abnormalities.
• Cell membrane surface area is decreased impairing
  the flexibility needed to pass through spleen's
  microcirculation ? hemolysis in spleen.
• MCV normal, MCHC is increased. Reticulocytosis of
  15 to 30 and leukocytosis are common.
• Increased osmotic fragility but normal in mild
  cases it may be normal unless sterile
  defibrinated blood is first incubated at 37 C
  (98.6 F) for 24 h.
• Direct antiglobulin (Coombs') test is negative.
  RBC autohemolysis is increased and corrected by
  addition of glucose.

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Treatment Prognosis

• Splenectomy is the only specific treatment
• Indicated in patients lt 45 yr with severe anemia
  (Hb lt 10 g), jaundice or biliary colic, or
  aplastic crisis
• Before splenectomy, the patient should be
  immunized with pneumococcal and Haemophilus
  influenzae vaccines.
• During splenectomy, a gallbladder with stones or
  evidence of disease should be removed. After
  splenectomy, symptoms usually abate, the RBC
  count rises, and the reticulocyte count returns
  to normal. Because spherocytosis persists, RBC
  fragility of the blood is still increased, but
  the patient is improved because the filter
  (spleen) for these abnormal cells is absent and
  the cells survive longer in the circulation.