ISSUE29ISSUE

October
2022

This issue
October Slides P.1
Monthly Digital Case Study P.1
Haemoglobin P.2-3
Cell Quiz P.2

October Slides
Slide 1
CLL.

Monthly Digital Case Study
Presentation October 2022, Slide 4

Slide 2
Nothing to report.

Slide 3
Nothing to report.

Slide 4See case study on the right.

FBC Results
WBC 21.2* (10^3/mm3)
RBC 2.51* (10^6/mm3)
HGB 7.4* (g/dL)
HCT 22.7* (%)
MCV 90 (fL)
MCH 29.5 (pg)
MCHC 32.6 (g/dL)
PLT 104* (10^3/mm3)

Blast Cell

Blast Cell

Slide 5
Leukemic phase of typicalMantle leukaemia.

Slide 6
Post-therapy follow-up ofAML7.

Hypo granularNeutrophil

Basophilic Stippling

Hypo Segmented(left Shift) Neutrophil

Nucleated Red BloodCell

Macrothrombocyte.

Promonocyte

Monthly Morphology Slide review (continued)
Quiz04 October 2022
Look closely at the slide below:

Male (78years old).
Clinical Haematology Unit. Hyposegmented (++) and hypogranulated(+++) neutrophils. Monocytes/Promonocytes/Monoblasts (+++).
Anaemia, Anisocytosis (++). Thrombocytopenia. AML post MDS?
Expert Comment: Pelger Neutrophils (+++), Macroplatelets.
Probable AML post MDS or AML probably with multilineage dysplasia.

Haemoglobin Structure and Function

Which of the below would you
associate with a film
appearance like this?
A) Sickle cellB) Hereditary ElliptocytosisC) Haemolysis

Last month’scell Quiz:

The primary function of Haemoglobin (Hgb) is to transport oxygen fromthe lungs to the tissues. The haemoglobin molecule consists of four
separate folded polypeptide chains. In normal adult haemoglobin thechains are named alpha and beta chains (known as HbA), which form ahydrophobic water repelling pocket around a heme group. The hemegroup is composed of a central Iron atom complexed to 4 Nitrogenatoms. Oxygen is capable of reversibly binding to the heme group. The
Haemoglobin molecule can exist in 2 states – a tense (T) state which has
low affinity for Oxygen and a Relaxed (R) state which has a high affinityfor oxygen. This transition from one state to another enables Oxygen to
be taken up in the presence of high oxygen concentration (lungs) andrelease oxygen in low oxygen concentration. Changes to the structure ofthe polypeptide chains of the haemoglobin molecule has a profoundeffect on the function of the haemoglobin.

The Haemoglobin molecule

Look closely at this red cell.
What is unusual about it andwhat could this indicate?

Sickle Haemoglobin HbS
Answer:Blood film shows a red cell witha cabot ring and a Howell-Jolly
body. In this case, the patient
was displaying a
myelodysplastic picture withabnormalities in all cell lines
and circulating megakaryocytes.

Inheritance of an abnormal sickle cell gene causes the production of HbS.
The mutation is on the HBB gene and causes a single amino acidsubstitution in the Beta chain, specifically replacing Glutamic Acid withValine at position 6 of the Beta chain (Glu6Val or E6V). The inheritance of
the abnormal gene is autosomal recessive, therefore it is possible to have
either heterozygous beta chains with the Haemoglobin being made up of
50% HbA and HbS (Sickle cell trait) or homozygous where haemoglobin isentirely HbS (sickle cell anaemia).

In the presence of a low oxygen concentration, the replacement of Glutamic Acidwith Valine causes the abnormal HbS sub-units to stick together and form long,rigid molecules that bends the red cell into a sickle like shape. Sickled red cells aredestroyed prematurely, causing anaemia which may require blood transfusion.
Red Cells containing HbS have increased adhesion which can cause the formationof heterocellular aggregates, which physically causes small vessel occlusion andresultant local hypoxia.

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Sickle Cells
BibliographyHemoglobin: Structure,
Function and Allostery - PMC(nih.gov)
HBB gene: MedlinePlus
Genetics
Sickle Cell Disease - StatPearls- NCBI Bookshelf (nih.gov)

Editorial Team
Kelly Duffy
Andrew Fisher
The chance of inheriting either sickle cell trait or sickle cell anaemia is dependent
upon the genetic makeup of the parents. If both parents have sickle cell trait,
then there is a 1 in 4 chance of offspring having homozygous sickle cell anaemia
a 1 in 2 chance of having sickle cell trait and a 1 in 4 chance of having normal
haemoglobin.
Sickle cell anaemia is most common in Africa, the Middle East, and India. Peoplewith sickle cell trait do have a relative resistance to Plasmodium Falciparum andare less likely to get the disease, have a lower parasite count, and are less likely todie.

About usPatients with sickle cell trait do not normally have any symptoms unless the bodyis under immense stress e.g. intense exercise or low oxygen environments.

HORIBA UK Limited
Kyoto Close
Moulton Park
Northampton, UK
NN3 6FL

Patients with Sickle Cell Anaemia start presenting with symptoms after about age
6 months and can present with many different symptoms including VasoOcclusive crisis, Acute Chest Syndrome, infections, stroke, PE, renal complication,and aplasia.

HORIBA Medical
Parc Euromédecine, 390
Rue du Caducée, 34790,
FrancePatients often have sickle cell crisis where there is acute pain caused by vaso
occlusions. A sudden drop in the already low Hb level may occur due to either
splenic sequestration, aplastic crisis (often in conjunction with parvovirus B19
infection or other vital infections), or hyper haemolytic crisis (particularly if thepatient is G6PD deficient).

www.horiba.com/medical