Case #1

A 15-year old black male who recently returned from the Philippines was diagnosed with malaria one week ago and started on primaquine. Three days ago, the patient noticed that his urine was dark brown. His mother said she had similar change in her urine when she was a child after a urinary tract infection was treated with nitrofurantoin. Physical exam of the patient reveals a heart rate of 104/minute with nail bed and conjunctival pallor. Laboratory work revealed hematocrit of 29 with macrocytic indices, as well poikilocytes, spherocytes, schistocytes, and bite cells in the peripheral smear. The platelet count was 190,000. His urinalysis revealed 4+ hemoglobin with positive iron stain.

I. Title: None indicated

II. Author: None indicated

III. Defnition of Keywords

Glucose-6-phosphate-dehydrogenase deficiency- an enzyme deficiency common in black people

Hemolysis – refers to the destruction of red blood cell.

Primaquine – antimalarial drug that induces hemolysis especially on G6PD deficient individuals.

IV. Clinical History:

A 15-year old black male who recently returned from the Philippines was diagnosed with malaria one week ago and started on primaquine

V. Physical Exam:

Physical exam of the patient reveals a heart rate of 104/minute with nail bed and conjunctival pallor.

VI. Laboratory Findings:. Laboratory work revealed hematocrit of 29 with macrocytic indices, as well poikilocytes, spherocytes, schistocytes, and bite cells in the peripheral smear. The platelet count was 190,000. His urinalysis revealed 4+ hemoglobin with positive iron stain.

VII. Patient's impression:

Susceptibility to drug-induced hemolytic anemia, presumably by the action of primaquine used to combat malarial infection, in glucose-6-phosphate dehydrogenase deficient individuals.

VIII. Discussion:

What is the diagnosis of the patient?

Susceptibility to drug-induced hemolytic anemia, presumably by the action of primaquine used to combat malarial infection, in glucose-6-phosphate dehydrogenase deficient individuals.

Give its pathophysiology.

In the case of G6PD deficient individuals, the person is prone to red blood cell hemolysis because there is no or little glucose-6-phosphate dehydrogenase that will regulate and control the production of oxygen radicals that are responsible for oxidative burst in cells. In other words, this enzyme is responsible for the protection of cell from oxidative damage by initiating the hexose monophosphate pathway which in turn leads to the production of nicotinamide adenine dinucleotide phosphate (NADPH) in red blood cells. The NADPH reduces the oxidize glutathione. The reduced glutathione, when utilized by an enzyme, removes peroxide from the erythrocyte. But with the side effect of primaquine, G6PD deficient individuals’ regulatory mechanism for oxygen radicals complicates the condition and put the affected person’s life at the risk of death, since the metabolites of primaquine induce production of oxyhemoglobin, which leads to severe hemolysis especially; redundantly citing, in G6PD deficient individuals, which I suspected in the case of this 15-year old black male.

What would be the possible treatment for the patient?

The possible treatment for the patient includes gradual withdrawal of primaquine after treating the malarial infection, and possible blood transfusion, specifically packed red blood cell, to compensate for the decrease in peripheral red blood cells

What was the cause why he experienced hemoglobinuria?

The cause of hemoglobinuria is the increase destruction of red blood cell thereby increasing the free hemoglobin values in the plasma which in turn, outnumbers the haptoglobin and other secondary hemoglobin carriers, when at excessive degree of hemolysis. Hence, some of the unbound hemoglobin find their way to the renal system to be flushed out and seen in urine. Thus, hemoglobinuria occurs.

If untreated, what will happen to the patient?

The patient will experience severe anemia due to drug –induced hemolysis and the toxic effects of hemoglobin deposition on various tissues, which include oxidation of various substances, which can be harmful to nearby tissue and cells at the site, thereby causing tissue damage and necrosis and other associated complications, for instance, nephropathy.

IX. Reference

Introduction to Hematology by Steninger.