Lab Case 72 – Interpretation

25 year old female with hereditary spherocytosis

Abnormalities and causes of presentation include:

– Evidence of haemolysis – low Hb, high LDH, bilirubin, ALP

– Possible sepsis – identify source (Pneumonia, Cholecystitis – high ALP/bilirubin, Pyelonephritis etc), however note that the inflammatory markers are normal so acute haemolytic crises must be kept in mind

Clinical Features of HS:




– Investigations in ED:

Mild to moderate anaemia

Blood film- spherocytes, increased reticulocytes, normocytic

Elevated bilirubin, LDH

Increased MCHC

Coomb’s test – is only positive in auto immune haemolytic anaemia

Osmotic fragility test is unreliable

Definitive Treatment:

– Splenectomy

Problems of Splenectomy:

– surgical risks

– Infections

Fatal sepsis caused by capsulated organisms (eg, Streptococcus pneumoniae, Haemophilus influenzae) is a recognized complication in children who have had a splenectomy. The estimated rate of mortality from sepsis in these children is approximately 200 times greater than that expected in the general population. Although most septic episodes have been observed in children whose spleens were removed in the first years of life, older children and adults also are susceptible.

Vaccination against pneumococcus and H influenzae must be administered to patients prior to splenectomy and, indeed, probably to all patients with severe HS. If a partial splenectomy is performed, splenic function is preserved and vaccinations may be delayed until after surgery; however, the long-term data are not well established.

– Vascular events




Pulmonary hypertension

Complications related to HS:

  1. Haemolytic crises
  2. Non haemolytic crises – folate deficiency, aplastic anaemia
  3. Pigment type Cholelithiasis
  4. No increase rate of rupture from trauma, increase infection risk post
  5. Parvovirus infection

Rapid haemolysis can be triggered by viral and bacterial infections causing jaundice. anaemia and tender splenomegaly

Aplastic crises due to viral induced bone marrow suppression

Megaloblastic crises are rare but can occur due to folate deficiency. Patients require folate supplementation due to increased erythropoiesis.




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