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Langerhans cell histiocytosis "granulomatous" (371787)

Langerhans cell histiocytosis "granulomatous"
Subtitle: NODE

Type:
B11-18610

Sender:
ugnius

2011-06-17 15:35
INCTR - EBMWG Hematopathology Online

STORY: 71 yrs old female with right sided lateral lymphadenopathy (4x6x7 cm confluent immobile nodes for 1 year). CRB elevated. Slight monocytosis is present.

CLIN DIAGNOSIS: "Lymphoma?".

HISTO: Confluent hypercellular and pleomorphic"epithelioid" granulomas with acellular necrosis ("caseating") with TBC like appearance.

IH: A lot of Langerhans cells within granulomas: CD1a+; CD68+; S100+ with admixture of histiocytes, multinucleated cells...

PROPOSED DIAGNOSIS: TBC granulomatous lymphadenitis (enriched LC) vs Langerhans cell histiocytosis: "granulomatous, TBC like pattern".

PROBLEMATIC ASPECTS: 1. The absense of Eos? 2. The theorethical possibility of association with other LPL (not present there?)?

Thank you for beeing together.

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ugnius 2011-06-18 12:10	The case is ready and open for discussion. Thank you.
vrugt 2011-06-20 10:01	Did you also perform ZN and Grocott stains?
ugnius 2011-06-20 10:20	At first glance ZN/Grocott/PAS negative... despite TBC granulomatous picture.
tzankov 2011-06-20 12:35	Dear Ugnius, intersting case. Why did you stain it for CD1a? The Ki-67 is rather high, I am wondering if you could stain for a B- and T-cell marker in order to exclude a lymphoma masked by a grnulomatous process. A ZN should be done in any case. I do not think that this is a LHC sensu strictu, rather a grnaulomatous process enriched in LC (infectious or para-neoplastic).
ugnius 2011-06-20 13:10	This biopsy has a right/standard way to be called granulomatous necrotic lymphadenitis... But I have mentioned "high cellularity" within granulomas (usuallt TBC granulomas are more "loose") with grooved nuclei: and the storry begans...
ugnius 2011-06-20 13:11	With or without IH any NHL cannot be seen around up to date.
vrugt 2011-06-20 17:32	Because of the absence of eosinophils (and to lesser degree also the age of the patient) I am a little bit reluctant with the diagnosis LCH, despite the striking positivity for CD1a and S-100. A CD1a+ dendritic cell hyperplasia as part of an inflammatory response should be considered as well. Do you know more about the clinical presentation?
ugnius 2011-06-29 16:29	Thank you. The patient redirected to hemato and hopefully we will hear smth new later...
diane.c.farhi 2011-07-08 15:48	Once again, I apologize for the lateness of this comment. I think this granulomatous lymphadenitis is more likely to be inflammatory than LCH. What about sarcoidosis with a rich population of Langerhans cells? I found a couple of old articles suggesting this possibility (Histopathology 1991;18:149-53, Scand J Immunol 1987;25:461-7). If infectious and other inflammatory etiologies are excluded, perhaps sarcoidosis should be considered. Has the patient had a chest x-ray? Is there any more clinical information?
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Last modified: 2011-06-28 12:47:32