International Journal of Celiac Disease, 2016, Vol. 4, No. 1, 1-3
Available online at http://pubs.sciepub.com/ijcd/4/1/5
© Science and Education Publishing
DOI:10.12691/ijcd-4-1-5
It's Counting That Counts
Michael N Marsh
*
Oxford University, UK
*Corresponding author: mikemarshmd@uwclub.net
Cite This Article: Michael N Marsh, “XIt's Counting That Counts.” International Journal of Celiac Disease,
vol. 4, no. 1 (2016): 1-3. doi: 10.12691/ijcd-4-1-5.
In the last issue of the journal, Siriweera and colleagues
[1] revisit the controversial area of inter-epithelial space
lymphocyte (IEL) "counting" in regard to the diagnosis of
celiac disease (or, as I prefer, for good reason, gluten
sensitivity). Their histologically-based approach is a
further attempt to re-determine the diagnostic validity of
this method of enumerating IEL. It was based on another
definition of the 'control' upper limit for normal mucosae,
but also to 'assess the diagnostic accuracy of existing
criteria for IEL in diagnosis of celiac disease'.
Briefly, based on x400 examinations of 3μm H&E
sections of formalin-fixed tissue, their upper limits for
control, and celiac, mucosae were 4/100 and 20/100
enterocytes, respectively. These values are exceptionally
low for controls, while the ranges for each series were not
given, so the extent of their overlap cannot be inferred.
Conversely, the data published from our laboratory [2],
based on strict morphometric criteria using oil-immersion
optics with 1μm toluidine blue-stained Epon sections
yielded 95% confidence limits of 5-27, and 14-61,
respectively. Despite that attention to detail, there was a
clear overlap between each nominal population, thus
reinforcing the obvious conclusion that there is no magical
"cut-off" point, because the lymphocyte response (like
weight, blood pressure, height, acid secretion) is a graded
characteristic [3]. That indicates that there never was, nor
could be, clear-cut divisions, as if the two IEL populations
(control and celiac) represented separate, bimodal
populations. The results of every study published are also
confirmatory of that truth, too, but is contrary to Lerner
and Matthias [4].
Apart from a few references, there was no in-depth
engagement by Siriweera with the considerable literature
on IEL, and one which still remains unresolved, as is clear
from the recent papers in this journal by Pena, and by
Lerner & Matthias (and see similar recent explorations in
Gastroenterology Hepatology: from Bed to Bench, 8(4),
2015). I was also rather surprised that Siriweera's counts
were made on 'uninterrupted sections of epithelium
comprising 500 enterocytes' which, in my experience,
would be very difficult to achieve for normal mucosae and
even more so for damaged specimens. Neither do they
grade their celiac mucosae (whether one believes the so-
called Marsh Classification [5] – albeit modified by so
many others - or not).
Now, that is very important because with the very early
lesions, histological diagnosis may be well-nigh
impossible, especially if the villi are tall and non-
infiltrated (Marsh 0). As I was both a practising
gastroenterologist and a laboratory-based investigator, it
was always clear to me that the histology is but an aid to
diagnosis, the latter being ultimately made, with all other
relevant data weighed, at the bedside by the clinician in
charge. Moreover, diagnosis is difficult in the early stages
of evolving celiac disease irrespective of the diagnostic
approach utilised [6]. This problem is diagnostically
magnified by the current epidemic of so-called non-
coeliac gluten sensitivity. This dilemma illustrates the
fragile confirmation of coeliac disease in its earliest stages
of development, and irrespective of the sophistication of
the laboratory tests employed. My own view is that
Siriweera's paper hardly contributes to this important
debate, and certainly does not help in clarifying any of the
issues arising.
So, what are the issues arising? Many have been nicely
revisited by Lerner & Matthias [4]: (i) effects of age on
IEL (ii) presence of infections or parasites (H pylori, Giardiasis)
(iii) genetic background (iv) geographic, environmental
and cultural influences (v) role of other methodologies,
including flow cytometry. These authors ask for a standardised
methodological approach for enumerating IEL, but that is
seemingly an impossible request, since as noted above,
there have been innumerable papers on this subject
without the slightest attention given towards a universally-
agreed approach. Neither the official guidelines published
by the American Gastroenterological Association nor the
British Society of Gastroenterology have elucidated a
preferred modus operandi. And as far as mucosal
classification goes, the BSG paper [7] recommends any of
the approaches available. One can conclude that neither
the authors invited, nor the Organisations' representatives,
acknowledged that this allegedly important histological
tool employed widely should have been standardised –
and accepted by everyone.
One recent advance which does require attention,
however, has seen the grouping of all mucosal specimens
(Marsh 0, I and II) into the category termed by Rostami as
"Microscopic Enteropathy" (The Bucharest Consensus)
[8]. Here it is recognised, and quite rightly, that a list of
differential diagnoses obtains for each mucosal category,
thereby overcoming the somewhat ridiculous view that,
for example, Marsh I and II lesions represent "non-
specific" appearances. But there is no such histological
entity as "non-specific": every tissue either reflects its
physiological or pathological state when sampled. It is
often scarcely recognised that the Marsh III lesion is