Investigations

1st investigations to order

FBC

Test
Result
Test

First test to perform. FBC shows anaemia (haemoglobin 40 to 100 g/L [4 to 10 g/dL], depending on severity), which is typically normochromic and normocytic/mildly macrocytic, with an increased MCHC.

Result

anaemia and normochromic red cell index

reticulocyte count

Test
Result
Test

Indicates marrow response to anaemia.

Result

reticulocyte percentage typically elevated to >0.10 (>10%) (degree of elevation proportional to the degree of anaemia)

urinalysis

Test
Result
Test

Dipstick testing detects urobilinogen and protein if intravascular haemolysis is present.

Result

haemoglobinuria

unconjugated (indirect) bilirubin

Test
Result
Test

Increased haem catabolism.

Result

elevated

lactate dehydrogenase (LDH)

Test
Result
Test

Elevated LDH as a consequence of RBC destruction and haemoglobin release.

Result

high

haptoglobin

Test
Result
Test

Binds free haemoglobin; low plasma values suggest intravascular haemolysis.

Result

low

peripheral blood smear

Test
Result
Test

Presence of blister or bite cells suggests oxidative stress as a cause of haemolysis. Heinz bodies (fragments of denatured haemoglobin) are seen in acute haemolysis.

Result

anisocytosis, abnormal forms, bite cells

Investigations to consider

G6PD fluorescent spot test

Test
Result
Test

Detects deficient production of reduced nicotinamide adenine dinucleotide phosphate(NADPH) from nicotinamide adenine dinucleotide phosphate (NADP).

NADPH is fluorescent and its absence (due to G6PD deficiency) results in lack of fluorescence.

Result

negative

G6PD spectrophotometry

Test
Result
Test

Spectrophotometric analysis of NADPH production from NADP quantifies enzyme activity. Quantitative spectrophotometric assay should be undertaken to confirm or exclude diagnosis if the screening test is abnormal or borderline.[26][33][34]

G6PD activity should be measured by quantitative spectrophotometric assay first line in female patients. A cytochemical test should be undertaken if quantitative assay results are intermediate or equivocal or if there is a clinical or genetic reason to suspect that a woman is heterozygous for G6PD deficiency.[26][33][34]

Result

reduced G6PD activity

molecular analysis

Test
Result
Test

Can be considered when initial tests are equivocal. Candidates for molecular analysis include heterozygous females, and males with Klinefelter syndrome (who may have intermediate G6PD activity).[26]

G6PD variants can be ascertained using polymerase chain reaction (PCR).[36][37][38]

Result

positive

Emerging tests

point of care testing

Test
Result
Test

Rapid diagnostic testing can be useful in certain situations at the point of care (e.g., neonatal screening programmes or to determine the safety of primaquine treatment in the context of Plasmodium vivax malaria control programmes), but availability may be limited.[40]

Result

negative

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