ISSUE

22

ISSUEFebruary
2022

This issue
Last Month’s Slides P.1
Monthly Digital Case Study P.1-2
Interesting Case/ P.2-3
Cell Quiz P.2
May –Hegglin Anomaly P.3

Last Month’sSlides
Slide 1
Patient hospitalized in Clinical
Haematology. Analyzer alarms:
Lymphocytosis/atypicallymphs. Monomorphic
hyperlymphocytosis associatedwith very many naked nuclei:
ProbableCLL. Immunophonotyping of
circulating lymphocytes
requested.

Slide 2
Neutrophilia. Eosinophilia.
Basophilia. Expert comment:
The morphology (plateletanisocytosis +++) requires aninvestigation for a chronic
myeloproliferative syndrome,with molecular research (Jak2,MPL, CalR and especially
Bcr_Abl). Myelogram + BMB.
Note a double erythrocyte
population.

Slide 3
See Case Study.

Slide 4
Intensive care unit andperioperative medicine.
Neutrophilia. Polycythemia.

Slide 5Macrocytic anemia:Anisocytosis (++). Macrocytosis(++). Hypersegmentation ofneutrophils (++). Expert
comments: Indicative ofvitamin deficiency ?

Slide 6
No clinical information.
Nothing abnormal detected.

Monthly Digital Case Study
January 2022 Slide 3
Presentation
3 January 2022 - Female, 20 years old, hospitalized in the gynecology
unit. Polymorphic lymphocytes. Expert comment: Aspect in favor of infectious
mononucleosis syndrome (lymphocytes with hyperbasophilic cytoplasm).

FBC Results
WBC 9.2 (10^3/mm3)
RBC 4.6 (10^6/mm3)
HGB 14.3 (g/dL)
HCT42.4 (%)
MCV 92 (fL)
MCH 31.1 (pg)
MCHC 33.8 (g/dL)
PLT 196 (10^3/mm3)
Neutrophils 34.5%
Lymphocytes 57.5%
Monocytes 7.1 %Eosinophils
0%Basophils
0%

Atypical Lymphocytes

Slide review
Expert comment: Aspect in favor of infectious mononucleosis syndrome(lymphocytes with hyperbasophilic cytoplasm). Lymphocytes are large, reactive cellswith an abundance of cytoplasm. The cytoplasm is often blue or a deep
blue colour.

Cell Quiz:The film below is from a patientwith Haemolytic Anaemia. Whatfeature do you see?

Slide 3 Review continued
Infectious Mononucleosis or glandular fever is caused by the Epstein-Barr Virus. It wascommonly known as “kissing disease” as it primarily effected young adults. The virus
targets the lymph tissue in the tonsils, infecting B-cells and inducing an immune
response.
FBC results typically show a moderate rise in the WBC, with an absolute lymphocytosis.
Large numbers of atypical lymphocytes are seen in the blood film (see above).
Possible Follow up tests:
Monospot Test is the standard to confirm the presence of heterophile antibodies, inconjunction with FBC results and blood film examination.

An interesting case presenting with Thrombocytopenia
A) Target cellsB) SpherocytosisC) Poikilocytosis

Last Month’s Cell
Quiz:
Name the cells highlighted in
the film below:

A 41-year-old female attending her General Practitioner with a history of epistaxis (nosebleeds) and slight bruising. A FBC was taken, and a full clinical history was taken. Thepatient was not taking any anticoagulation or Aspirin. The FBC results were as follows:
WBC 3.8
RBC 5.14
HGB 14.9
HCT 43.3
MCV 84
MCH 29MCHC 34.4
PLT 23
MPV 13 fL(10^3/mm3)(10^6/mm3)(g/dL)
(%)
(fL)
(pg)
(g/dL)(10^3/mm3)

The result indicated Thrombocytopenia with large platelets present (MPV high). A bloodfilm was requested and confirmed the presence of very large Platelets as well as spindle
like inclusions (Döhle bodies) in the cytoplasm of Granulocytes and Monocytes.

Platelets

Answer:The cells indicated in the film by
the blue arrows are Sickle Cells.
These are red blood cells whichhave "sickled" forming thecharacteristic crescent shape.
Sickling occurs when
Haemoglobin-S is deoxygenated,
forming long polymers whichcross-link and cause the cell tosickle. The abnormality is due toa defect in the B-haemoglobin
chain of the haemoglobin
molecule due to a substitution ofvaline for glutamic acid in
position 6 of the B-chain.
The shape change weakens thecells, promoting prematuredestruction, known as
intravascular haemolysis, whichleads to anaemia. This also leads
to free haemoglobin in theplasma, which is then bound by
haptoglobin. A low haptoglobin
level can indicate intravascularhaemolysis.

Neutrophils showing the presence of Döhle bodies

A presumptive diagnosis of May-Hegglin Anomaly was madeQSP 2.0
May – Hegglin Anomaly (MHA)
MHA is the most common cause of inheritable giant platelet disorders and shows
autosomal dominance inheritance and was first described by May in 1909 and later byHegglin in 1945. Even though the actual platelet count is low not all patients presentwith bleeding. Since the Mean Platelet Volume is exceedingly high the actual circulating
biomass of platelets is within the normal limits. The number of Megakaryocytes in thebone marrow is not increased and their volume is normal. Approximately half of thereported patients are asymptomatic but the other half have platelet counts less than 50
x10^3/mm3 and have abnormal bleeding in the form of epistaxis, gingival bleeding,easy bruising, menorrhagia, and excessive bleeding post surgery. MHA patients may
complain of some non haematological symptoms, such as high-tone hearing loss, renal
involvement, and cataracts.
The frequency of MHA is very low and as of about 10 years ago only 170 cases werereported in the literature. It occurs most often in people of Greek or Italian descent.
MHA is caused by a genetic defect in the MYH-9 gene which is present at chromosomal
region 22q12-13 and codes for non-muscle myosin heavy-chain IIA. Disorders ofproduction of myosin heavy-chain IIA affect megakaryocyte maturation and platelet
fragmentation causing the macrothrombocytes seen. The Döhle bodies seen should not
be confused with the large azurophilic granules seen in Neutrophils of patients withsevere infections. The Döhle bodies seen in MHA patients are composed of shortsegments of Rough Endoplasmic Reticulum, clusters of Ribosomes and a framework ofparallel filaments.

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Bibliography
May Hegglin Anomaly - anoverview | ScienceDirectTopics
Essential Haematology
A.V .Hoffbrand & J.E.Pettit

Editorial Team
Kelly Duffy
Andrew Fisher

MHA is 1 of 4 overlapping syndromes that all have in common a mutation on the MYH-9
gene, the other disorders being Epstein syndrome Fechtner syndrome, and Sebastianplatelet syndrome. They all have in common the prescence of macrothrombocytes withSebatian and Fechtner having the largest. MHA has both macrothrombocytes and large
leucocyte inclusion (Döhle) bodies, whereas small leukocyte cytoplasmic inclusion
bodies are observed in Fechtner syndrome and Sebastian platelet syndrome. No
inclusion bodies can be seen with standard light microscopy in Epstein syndrome
patients.
Since MHA is a genetic disorder family studies may reveal other family members
unknowingly having MHA. Since bleeding episodes are rare in MHA patients platelettransfusion is rare and only indicated as and when necessary.
Other disorders associated with giant platelets are:Bernard -Soulier Syndrome (BSS) – autosomal recessive disease with quantitative or
qualitative defect of platelet GPIb-IX-V receptor. The platelet has reduced or noresponse to Ristocetin in platelet aggregometry tests. Patients have mild to moderate
bleeding and require platelet transfusions for bleeding and surgery.
Platelet Type Von Willebrand disease is an autosomal dominant disorder resulting in again of function mutation in the GPIbα receptor causing increased binding of platelets
to Von Willebrand Factor and platelet clumping. The response of platelets to Ristocetin
is increased.
Mediterranean Macrothrombocytopaenia is an autosomal dominant disorder withmutations in GP1BA resulting in mild macrothrombocytopaenia.
Grey Platelet Syndrome is characterised by the absence of platelet alpha granules andcan also result in large platelets with platelet dysfunction.

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