Case study #6 –

25-year-old Japanese man was admitted to the hospital because of fever (37.8°C), sore throat,
and general malaise for several days. The color of his urine was unusually dark. The physician
noted anemic conjunctiva (delicate membrane lining the eyelids and covering the eyeballs),
cervical lymphadenopathy (swelling of the lymph nodes), and mild splenomegaly (enlargement
of the spleen).
Hematological data showed increased reticulocytes (7.8%; normal is <2.4%) and leukocytosis
with increased lymphocytes. The blood film showed moderate anisocytosis (variability of size of
erythrocytes), increased spherocytes (small, globular erythrocytes without the usual central
pallor), and a scatter of atypical lymphocytes.
The urine examination revealed no abnormality except an increase of urobilinogen.
Biochemical data for his serum showed total bilirubin levels of 99.2 μmol/L (normal is 3.4–20.5
μmol/L), conjugated bilirubin 15.4 μmol/L (normal is 0–6.8 μmol/L), alanine aminotransferase
125 U/L (normal is 0–40 U/L); and lactate dehydrogenase 570 U/L (normal is 120–250 U/L).

Biochemistry Assignment
Answer the following questions:
1. What was the most likely condition of this patient? (3 marks)
 The most likely condition of this patient is “Hereditary Spherocytosis with
Gallstones” as the physician noted anemic conjunctiva and a darker urine color this
is commonly observed in patient with nonspecific Viral Infections. Adults who
remain undiagnosed usually have a very mild form, and their HS remains undetected
until challenged by an environmental stressor.

2. What are the essential findings for the clinical diagnosis of this condition? (4 marks)
 The most diagnostic features of Hereditary Spherocytosis are:
Congenial hemolytic anemia
Spherocytosis on the blood film
Hyperbilirubinemia
 Gallstones of the pigment type, resulting from excess unconjugated bilirubin in bile,
may be found in very young children, but the incidence of gallstones increases
markedly with age. In patients with mild HS, cholelithiasis may be the first sign of an
underlying red cell disorder.
3. What is the treatment strategy? (2 marks)
 Folic acid is required to sustain erythropoiesis (to prevent Megaloblastic Crisis)
 Splecnectomy is the definitive treatment for Hereditary Spherocytosis. It usually
eliminates Hemolysis and the associated signs and symptoms.
 Generally, the treatment of HS involves presplenectomy care, splenectomy,
and management of postsplenectomy complications.
4. Depict the biochemical process that would have resulted in the noted results. (6 marks)

Histology Assignment
Describe the organization of the impaired structure that resulted in this clinical presentation.
Referenced diagram(s) may be included. (10 marks)