[CIS PIDD] [cis-pidd] Adenopathy in patient with Hyper-IgM syndrome

[Wall, Luke]

Just curious if these patients have been tested for NEMO? I'm assuming there is no epidermal dysplasia present... but NEMO is still a possible diagnosis given the class-switch recombination defect in a male demonstrating susceptibility to Pseudomonas, S. aureus, encapsulated bacteria, Salmonella, and viruses (I'm combining aspects of both Howard's patient and Prescott's patients... but perhaps NEMO could be considered in all three)? Although all testing for atypical mycobacteria was negative, the patient with the fistulous tract formation still seems to be behaving clinically like atypical mycobacteria infection???


Luke



Luke Wall, MD

Assistant Professor, Pediatrics

Section of Allergy Immunology



Louisiana State University and Children's Hospital

200 Henry Clay Ave. Research Bldg, Ste. 4228

New Orleans, LA 70118

504-896-9589 phone 504-896-9311 fax



________________________________
From: Prescott Atkinson, M.D. [PAtkinson at peds.uab.edu]
Sent: Thursday, August 22, 2013 6:04 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] Adenopathy in patient with Hyper-IgM syndrome

Hi Howard: I am following a similar patient – he has had recurrent massive cervical lymphadenitis with positive cultures on two separate occasions with Pseudomonas aeruginosa – no systemic spread and no other regional lymphadenopathy.  It responds on each occasion to appropriate antibiotic therapy but we have been unable to keep it from recurring.  He’s currently on an 8 week regimen of high dose cipro and is again responding.  I suggest aggressive, prolonged empiric antibiotic treatment for the pathogens you have previously cultured.  I actually have a second patient with a similar phenotype – both have declining T cell as well as B cell numbers, markedly elevated IgM and little or no IgA, IgG or IgE.   The first patient has had persistent, low-level EBV viremia also but this does not seem to be a primary player in his lymphadenopathy. The second patient suffered a prolonged gastrointestinal infection with Salmonella and developed intra-abdominal lymphadenopathy that was so severe that he developed symptoms from IVC compression.
Prescott

From: Howard Lederman [mailto:hlederm1 at jhmi.edu]
Sent: Thursday, August 22, 2013 10:08 AM
To: CIS-PIDD
Subject: [cis-pidd] Adenopathy in patient with Hyper-IgM syndrome

I need help with a patient hyper-IgM patient who has a necrotizing lymphadenitis.
M.K. is a 16 y/o male with hyper-IgM syndrome of undetermined etiology.  He has had persistent bilateral cervical lymphadenopathy since 6/2012.   Lymphadenopathy first was noted on a routine follow-up exam. A fine needle aspirate of a right cervical node during the summer of 2012, found pus and cultures grew Hemophilus and MRSA.  He was treated with clindamycin and Augmentin for 4 weeks, and then Bactrim for an extended course,  but there was only a slight clinical response.  Pathology from the FNA revealed histiocytic necrotizing lymphadenitis with a mixed lymphoid population.
On 8/29/2012, he underwent an excisional biopsy of 2 large lymph nodes in his right neck.  Pathology showed patchy geographic necrosis. There was no significant apoptotic debris or histiocytes in the necrotic areas which had an almost infracted look. There were abundant histiocytes rimming the necrotic areas. There were few, if any, neutrophils.  The architecture of the lymph nodes was distorted by this necrosis and histiocytes. Reed Sternberg cells or variants were not appreciated.  There were no granulomas identified and the paracortical infiltrate did not resemble viral change. PCR for EBV was negative.  There were scattered microcalcifications. CD68  and MPO staining highlighted abundant histiocytes.  Flow cytometry showed no evidence of lymphoma. AFB and GMS stains showed no evidence of microorganisms.  The morphologic features and immune-phenotype of the node raised the possibility of Kikuchi disease, however, lack of histiocytes and apoptotic bodies inside the necrotic areas was not considered to be consistent with Kikuchi disease.
The area of those biopsies never healed.  Other lymph nodes enlarged, there was a thin, yellow discharge.  The overlying skin broke down, and a sinus tract formed.  A PPD and a Quantiferon-Gold test were negative.  He was treated with prednisone (80 mg or approximately 1 mg/kg/day) with no response.  Enbrel was added, again with no significant clinical response.
In 3/2013, the site of the previous biopsies was opened open for debridement.  There was a large amount of “necrotic, fibrinous, cheesy material” with no obvious intact lymph nodes. He needed months of wound packing for the area to heal, and there continues to be some thin, yellow drainage through the incision sites.   Coagulase negative staphylococcus and Hemophilus influenzae were cultured.  He was treated with antibiotics despite the fact that we thought these organisms were contaminants transmitted from skin into the tissue via the sinus tract.   Prednisone was tapered and Enbrel was discontinued with a slow increase in the size of the lymph nodes
In 7/2013, he had an excisional biopsy of a left cervical lymph node.    Pathology was similar to what was seen last year.  No organisms identifiable by stain.  Coagulase negative staphylococci grew from a culture.  The following week, a culture of wound drainage grew enterococcus.  Once again, he was treated with antibiotics without response.
I have had hyper-IgM patients who develop generalized lymphadenopathy with follicular hyperplasia that usually responds to Rituxan.   This patient has a very different picture for which therapy has been ineffective and for which I do not even have a theory about the pathophysiology.  I wonder if anyone has seen this before, and/or if anyone has an idea for further elucidating the problem or an idea for empiric therapy.
I can email photomicrographs to anyone who wants to see them, but don’t think that I am allowed to include an attachment as part of the ListServ distribution.

Howard
Howard M. Lederman, M.D., Ph.D.
Professor of Pediatrics, Medicine and Pathology Division of Pediatric Allergy and Immunology Johns Hopkins Hospital - CMSC 1102 600 N. Wolfe Street Baltimore, MD 21287-3923
Phone: 410-955-5883
Fax: 410-955-0229
Email: Hlederm1 at jhmi.edu<mailto:Hlederm1 at jhmi.edu>

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