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Alinity hq

SICKLE CELL DISEASE

Description

This patient was diagnosed with sickle cell disease. The CBC showed decreased RBC, HGB, and HCT results, low MCV, elevated RDW and significantly increased % Reticulocytes. The rstRBC and RBC Frag flags were present. As a result of the RBC Frag flag, RBC and PLT counts as well as related parameters were suspect.

 

The RBC volume histogram showed an asymmetrical distribution with widening on the left side that indicates the presence of smaller sized RBCs. This corresponds with the low MCV value and the increased RDW. The IAS vs ALL scatter plot showed the presence of a gray population that represents lyse-resistant RBCs. The WBC count was not impacted by the presence of this cell population and was valid. The ALL vs IAS3 scatter plot showed the presence of a “rain shower” pattern of the RBC population (red) due to presence of irreversibly sickle cells in the blood.

 

Review of the peripheral smear confirmed the presence of sickle cells and RBC fragments and also described several target cells.

 

Sickle cell disease (SCD) is an inherited red cell disorder, which includes heterozygous sickle cell trait (with RBCs containing HbAS), homozygous sickle cell disease (with RBC containing HbSS), and a range of mixed heterozygous hemoglobinopathies. In conditions of low oxygen tension, the HbS polymerizes and forms rod-like structures causing RBCs to become hard, sticky, and c-shaped (sickle) in the circulation (Wahed, CDC). Polymerization of HbS in SCD can result in RBCs becoming resistant to lysis and sphering during CBC analysis.

Numerical ResultsSuspect

 
WBC 13.2 10e3/μL
NEU 6.13 10e3/μL 46.6 %
LYM 4.98 10e3/μL 37.9 %
MONO 1.51 10e3/μL 11.5 %
EOS .208 10e3/μL 1.58 %
BASO .269 10e3/μL 2.04 %
IG .059 10e3/μL .445 %
NRBC .148 10e3/μL
NR/W 1.12

 
RBC 2.81 10e6/μL rstRBC
HGB 7.41 g/dL
HCT 21.6 %
MCV 76.6 fL
MCH 26.3 pg
MCHC 34.4 g/dL
RDW 23.3 %

 
RETIC 207. 10e3/μL 2.44 %
IRF .482
MCHr 27.0 pg

 
PLT 467. 10e3/μL RBC Frag
MPV 7.76 fL
%rP 2.78 %

 
FIGURE 1. IAS X ALL (WBC):
Intermediate angle light scatter vs. size plot
In addition to the WBC subpopulations, there is presence of a gray population that represents lyse-resistant RBCs. The presence of lyse-resistant RBCs corresponds to the presence of sickle cells and target cells.
FIGURE 2. ALL X IAS3 (RBC/PLT):
Size vs. intermediate angle light scatter
The RBC populations shows a “rain shower” pattern instead of a compact population seen in normal subjects, due to abnormal light scattering properties of sickle cells.
FIGURE 3. IAS2 X ALL (RBC/PLT):
Intermediate angle light scatter vs. size plot
The left lower portion of the RBC population (red) likely represents the fragmented RBCs that are identified by the analyzer with the RBC Frag flag. While there is a close proximity of the RBC population (red) to the PLT population (yellow) due the presence of smaller RBC fragments, a clear separation is observed between the two cell populations.
Alinity hq Casebook. Sickle Cell Disease. ADD-00061876-v3 2020. p.34-35

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