ISSUE10ISSUE

February
2021

This issue
Last Month’s Slides P.1
Monthly Case study Slide 2 P.1
Hereditary Spherocytosis P.2-3
Morphology Tip P.3

Last Month’sSlides
January 2021 Slide
Summaries
Slide 1
Circulating follicularlymphoma. Monomorphic
lymphocytosis consisting of small
lymphocytes with a highnucleocytoplasmic ratio and anucleus sometimes notched "incoffee beans".

Slide 2
Mantle cell lymphoma

Slide 3
Follow-up of a hereditaryspherocytosis.
Anisocytosis (++). Polychromasia(++). Spherocytes (+++). (See articleon page 2)

Slide 4Multiple alarms on analyser.
Blood blastosis.

Slide 5
Nothing abnormal to report.

Slide 6
Nothing abnormal to report.

Monthly Digital Case study
Slide 2Presentation
02 January 2021 Male (76 years old) URGENT:
Exploration of a hyperlymphocytosis.
Immunophenotyping of circulating lymphocytes
in progress.

FBC Results
WBC
RBC
HGB
HCT
MCV
MCH
MCHC
PLT

27.5 (10^3/mm3)3.91 (10^6/mm3)
10.3 (g/L)
35.0 (%)
90(fL)
26.3 (pg)29.4 (g/dL)
124 (10^3/mm3)

Neutrophils 13 %Lymphocytes 8.3 %Monocytes 0.9 %Eosinophils 0.9 %Basophils
0.0 %Slide review
Cytology: Rather large cells, with high nucleocytoplasmic ratio, nucleus with anirregular outline (notches, foliate appearance) and with a rather basophilic
cytoplasm.

Diagnosis
Mantle Cell Lymphoma.
Mantle cell lymphoma (MCL) is a distinct subtype of mature B-cell non-Hodgkin
lymphoma (NHL) that accounts for 5–10% of all NHL. MCL is generally anaggressive NHL with characteristic morphology andimmunophenotype, which typically presents in elderly
patients with advanced disease and shows a poor prognosis.

Hereditary
Spherocytosis

Monthly Morphology
Quiz:An overview of the clinicalfeatures, laboratory findings
and treatment
QSP case 3 slide showing spherocytic red cells

Introduction
Hereditary Spherocytosis (HS) is a heterogenous group of disorders
characterised by the presence of Spherocytes, Clinical features may include
What is the name for the anaemia, jaundice, reticulocytosis and splenomegaly.

cell in the red box and what
condition may you see them Clinical Presentation
03 January 2020
in?

Last month’s Quiz:
Who first discovered
haemoglobin?

Female (45 years old)
WBC 5.5 (10^3/mm3)
RBC 3.37 (10^6/mm3)
HGB 10.5 (g/dL)
HCT 30.5 (%)
MCV 91 (fL)
MCH 31.2 (pg)
MCMH 34.4 (g/dL)
PLT 183 (10^3/mm3)
Follow up of Hereditary Spherocytosis

QSP case 3 slide showing spherocytic red cells

Hereditary Spherocytosis (HS)Hereditary Spherocytosis (HS) was first described in 1871 and the first recorded
splenectomy performed soon after. HS shows a wide range of features inregard, severity, mode of inheritance and the actual cause of the protein
defect. It is a relatively common disorder with an incidence in North Europeand North America of between 1 in 2000 to 1 in 5000 (depending on method ofanalysis). Affected patients will show a variable degree of anaemia, jaundice,reticulocytosis and splenomegaly with the clinical severity ranging fromsymptom free (carrier) to severe haemolysis. Exacerbation of clinical severity
The haemoglobin compound can be caused by illnesses such as Infectious Mononucleosis. The majority
was discovered in 1840 by (75%) of cases will have a family history even if HS has not been formallydiagnosed e.g. anaemia, splenectomy, gall stones, jaundice. The diagnosis of HS
Friedrich Ludwig Hünefeld.
has been known to be made from birth all the way up to people in their 80’s90’s. Sometimes the diagnosis is made serendipitously. HS carriers may notshow anaemia, jaundice or splenomegaly and with no spherocytes seen on theblood film.

Red Cell Morphology
Normal red cells are biconcave discs which have a large surface area to volume
and have no nucleus, allowing the red cell to contain relatively large amountsof Haemoglobin. Red cells need to be flexible in order to travel through tiny
capillaries so that they can absorb and deliver oxygen where needed. A
disturbance in the cytoskeleton can have a profound effect on the flexibility
and eventual life span of the red cell.

QSP 2.0

Red Cell Morphology Continued
Red cell membrane integrity is dependent upon several proteins withspectrin being one of the main contributors. Abnormal red cell morphology
seen in HS is due a specific genetic mutation leading to a deficiency of, or adysfunction in spectrin, ankyrin, band 3 and or protein 4.2. The defectmeans that the red cells can not retain their biconcave shape and become
spherocytes.
Spherocytic red cells are removed from circulation by the spleen resulting in
splenomegaly, anaemia and an increase in bilirubin.

Diagnosis
Spherocytes can be distinguished from normal red cells by the uniform
dense colour of the cytoplasm and can be smaller than that of a normal redcell.
As described above, the clinical picture in HS is varied but if Spherocytes areseen in the blood film a Direct Antiglobulin Test (DAT) should be performed
to exclude auto haemolysis. A reticulocyte count should also be performed
to confirm the degree of excess red cell production.
A family history is essential in the correct diagnosis of HS with note being
taken of family members who have had a splenectomy for no obvious
reason or gall bladder problems.
Additional confirmatory tests, such as Osmotic Fragility, Acidified Glycerol
Lysis time (AGLT) and Pink test ( a modified AGLT) are all based on the factthat spherocytosis are more prone to lysis than normal red cells.

Disease Management
Folate therapy is recommended in children with moderate and severe HS.
The patient should be offered a regular review particularly those withmoderate to severe form as splenectomy may be required.

This Month’s Top Morphology Tip
Eosinophils and basophils are myeloid cells, formed during haematopoiesis.
Although both are granulocytes, they each have different characteristic whichallow you to identify them within a film:

Available as a single
use license and asite license whichallows up to 10
concurrent users.
To find out more,contact us.

BibliographyBlood Cells A practicalguide – Barbara Bain
Guidelines for thediagnosis andmanagement of
Hereditary Spherocytosis
A BMJ Guideline
publication

Guidelines for thediagnosis andmanagement ofhereditary spherocytosis,
bjh guideline

Editorial Team
Kelly Duffy
Andrew Fisher

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

Eosinophil• Eosinophils are 12-17uM in diameter• They have TWO LOBES• Large acidophilic granules whichstain bright red (sometimes
connected giving the appearance ofan old telephone)• 1-6% eosinophils in circulating WBCBasophil• Basophils are 10-14uM indiameter• Bean Shaped Lobes• Prominent dark blue/purple
staining of granules in basic stains• 0.5-1% of WBC’s- basophils are
not commonly noted in a normal
film

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