[PAGID] Nodular lymphoid hyperplasia

[Berger, Melvin]

I don't like it when these patients start getting hectic fevers. I have had one with a similar story in the liver initially negative on biopsy, then large B-cell lymphoma on repeat biopsy a couple of weeks later. At that point, the liver began increasing in size with each passing day. Responded well to CHOP and Rituxin. Consider a PET and/or repeat biopsies. 
 
Melvin Berger, M.D., Ph.D.
Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237
 
Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Abraham, Roshini S., Ph.D.
Sent: Wed 6/24/2009 5:34 PM
To: pagid at list.clinimmsoc.org
Cc: Park, Miguel A., M.D.
Subject: [PAGID] Nodular lymphoid hyperplasia



Clinical scenario:

23-year-old man perfectly healthy before the onset of symptoms starting 8 months ago presented with the following symptoms:

1.      follicular nodular hyperplasia shown on pathology after right hemicolectomy

2.      Night sweats drenching clothes and temperatures of 99.5-100.5 F on a daily basis.  

3.      Diarrhea under control but significant abdominal pain. 

 

Lab evaluations:

1.      Positive test:

a.      BAFF level obtained April 6, 2009, has now returned and is elevated in the 3433pg/ml range with normal being roughly 551 to 1775pg/ml.  

b.      Pathology showed marked follicular nodular lymphoid hyperplasia with polytypic B cells among other lymphocytes involving ileum, ileocecal valve, appendix and multiple regional lymph nodes.  Negative for malignancy.  

2.      Negative test:

a.      QuantiFERON, HIV, stool cultures, fungal serologies, Giardia evaluation, cryptosporidium evaluation, entamoeba histolytica evaluation, and CMV testing.  

b.      PET scan normal

c.      IgA, IgM, IgG normal and normal serum protein electrophoresis 

d.      T, B, NK cell numbers normal before rituximab

e.      CRP and sedimentation rate normal

f.        ALPS screen negative

g.      ANA, Rheumatoid factor, SS-A and SS-B ab, Jo 1 ab, Scl 70 ab, ds DNA all negative

h.      Neutrophil oxidative burst negative

 

Treatment to date:

1. prednisone 50 mg per day for two weeks January 2009 without improvement.

2. Rituximab therapy 375 mg/m2 four doses 1/26/09 - 2/16/09; incrementally increased abdominal pain 

3. Five-day course of high-dose Solu-Medrol 2 g/m2 completed 3/10/09; abdominal pain and night sweats seem to increase during the high-dose Solu-Medrol therapy.  

4. metronidazole ten-day course without clear improvement in intestinal symptoms or diarrhea. 

5. ciprofloxacin to treat possible bacterial overgrowth 3/12/09; stools decrease from five loose stools a day to three loose stools a day.

 6. anakinra starting 3/15/09 - 3/28/09 without clear benefit and subsequent discontinued. 

 7. cholestyramine for the diarrhea and Entocort (budesonide) with opening of the capsules prior to swallowing.  The patient is also adhering to a gluten-free diet at present.  His diarrhea improved, and he was able to eventual taper off of the cholestyramine.  

 

Question: 

Has anyone seen a patient with similar constellation of symptoms?  Any suggestions or advice regarding treatment or further testing?

 

Thanks,

Roshini Abraham

Miguel Park, MD

 

 

 

Roshini Sarah Abraham, Ph.D., D(ABMLI)

Consultant, Div. of Clinical Biochemistry & Immunology
Associate Professor of Medicine & Lab. Medicine & Pathology 
Director 
Cellular and Molecular Immunology Laboratory 
Department of Laboratory Medicine and Pathology 
Hilton 210 e 
Mayo Clinic 
200 1st St SW 
Rochester, MN-55905 
Ph: 507-266-9292 
Ph (Secy): 507-284-4055 
Fax: 507-266-4088 

 



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