Examine the following haematology data set.

Test

Value

Normal Range

WBC

9.5 x 109/L

(4.0 – 10.5)

RBC*

3 x 1012/L

(4.3 – 5.7)

Hb*

88 G/L

(130 – 170)

PCV*

0.36

(0.38 – 0.49)

MCV*

103fL

(83 – 98)

MCH

30 pg/L

(28 – 33)

MCHC*

370

(330 – 360)

Platelets

190 x 109/L

(150 – 400)

Reticulocytes 5%, Occasional erythroblast, spherocytes, Heinz bodies +++

a)  Provide an explanation for the above set of results.

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College Answer

a)  Provide an explanation for the above set of results.

•    A high reticulocyte count, a high MCV are indicative of hemolysis
•    Heinz bodies indicate oxidative stress
•    Heinz bodies are seen in the setting of G6PD deficiency and drugs such as primaquine, in alpha thallasemia, chronic liver disease and splenectomy

Discussion

Heinz bodies, also known as Heinz-Ehrlich bodies are collections of denatured haemoglobin within the red cells, associated with the following conditions:

Increased oxidative stress due to toxins

  • Primaquine
  • Toxic solvents, eg. aniline, benzene, naphthalene
  • Quinidine
  • Dapsone toxicity

Unstable haemoglobins

  • Chronic liver disease
  • Alpha-thalassaemia
  • Certain congenital "Heinz body anaemias"
  • Methylene blue methaemoglobinaemia
  • Dapsone-induced methaemoglobinaemia

Deranged RBC metabolism

  • Glucose-6-phosphate dehydrogenase deficiency
  • Trimethoprim-sulfamethoxazole (Bactrim)

Decreased clearance of defective RBCs

  • Post splenectomy

Thus, these bodies form in oxidative stress of toxic exposure, with unstable haemoglobin subtypes, and in context of deranged RBC metabolism. In this case the cells are also macrocytic, and there seems is a reactive increase in reticulocyte count. Does this scream "haemolysis"? Probably not. So, what could have caused such a picture? G6PD deficiency is usually associated with macrocytosis, and haemolytic anaemia, but rarely with spherocytosis.

References

References

HARLEY, JOHN D., and ALVIN M. MAUER. "Studies on the formation of Heinz bodies. II. The nature and significance of Heinz bodies." Blood 17.4 (1961): 418-433.

JANDL, JAMES H. "The Heinz body hemolytic anemias." Annals of internal medicine 58.4 (1963): 702-709.

FERTMAN, MANUEL H., and Mildred B. Fertman. "Toxic anemias and Heinz bodies." Medicine 34.2 (1955): 131-192.

Selwyn, J. G. "Heinz bodies in red cells after splenectomy and after phenacetin administration." British journal of haematology 1.2 (1955): 173-183.

GOLDSTEIN, BERNARD D. "Exacerbation of dapsone-induced Heinz body hemolytic anemia following treatment with methylene blue." The American journal of the medical sciences 267.5 (1974): 291-297.

Coleman, M. D. "Dapsone: modes of action, toxicity and possible strategies for increasing patient tolerance." British Journal of Dermatology 129.5 (1993): 507-513.