[PAGID] lymphopenia

Tue Jun 20 15:21:24 EDT 2006

Here is the saga of one of our patients.  We have lately considered him
to have primary CVID with secondary "Castleman's", rather than thinking
his initial hypogammaglobulinemia was Castleman's-related, in keeping
with a sense of the heterogenous nature of this process.  T cell number
and function have been OK on multiple occasions.  He has periodic
lymphocytosis with up to 85% B cells.  His complications have been
treated as they arise; we've not yet used extensive chemotherapy.  We
have talked for a few years about HHV8 testing but not arranged.

1991 (age 3 y) - presented for tonsillectomy due recurrent URTI, found
to have generalized adenopathy (chest, mediastinal, retroperitoneal),
hepatosplenomegaly and low platelet count (129,000 dropping to
15-30,000).  IgA deficiency and low IgG noted.  Node Bx ("Castleman's");
liver Bx (Granulomatous hepatitis)

1993 (age 5 y) - lung biopsy "consistent with "Castleman's disease";
splenectomy for thrombocytopenia

1994 (age 6 y) - pneumococcal septic shock 2d post myringotomy (had
Pneumovax, on Pen prophylaxis) 

1995 (age 7 y) - chickenpox;  "shingles" in 1996, 1999, June '00,
October '00, '01, November '03.

1996 (age 8 y) - arthralgia. Rheumatology evaluation: neg. ANCA, ANA,
Vwf.

1997 (age 9 y) - asthma develops; Tx inhalers.  ALPS testing Neg: "FASR
expression normal; incr. DN T cells; N apoptosis"

1998 (age 10 y) - pityriasis rosea; pseudomonas cellulitis

2000 (age 12 y) - CT: worse lung disease, multiple pulmonary nodules,
incr. nodes.  EBV load (-) ; SAP (N)

2004 (age 16 y) - recurring episodes of asthma/pneumonia; started on
IVIg infusions, little obvious effect

If folks think we have therapeutic options or should do other
investigations, we are open to suggestions.  Despite the story, this is
a big, muscular judo-black belt kid.

Anne Junker, MD
Associate Professor, Division of Infectious & Immunological Diseases,
Medical Director, Pediatric Specialty programs at BC Children's Hospital
Associate Director (Clinical), Child & Family Research Institute
Room K4-223
Children & Women's Health Centre 
4480 Oak Street, Vancouver, BC   V5H 3V4
Ph: 604-875-3591  Fx: 604-875-2414  email: ajunker at cw.bc.ca

	-----Original Message-----
	From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Kathleen E.
Sullivan
	Sent: Tuesday, June 20, 2006 11:30 AM
	To: pagid at list.clinimmsoc.org
	Subject: Re: [PAGID] lymphopenia

	I have seen two cases of Castleman's with what we are pretty
sure was a secondary immunodeficiency (ALC normal prior to symptoms). I
am very sad to report, both patients died after traditional
chemotherapy. 

	On Jun 20, 2006, at 2:20 PM, Christine Seroogy wrote: 

		Colleagues, 

		I have an update on the patient I presented last week
and, again, welcome opinions in terms of management.  The pathology on
the GI mass is now finalized as Castleman's disease.  Clinically, he
does not fit into unicentric or multicentric, but falls somewhere in
between.  It is CD20 negative and he is HHV8 positive.  So my heme/onc
colleagues are planning on treatment with chemotherapy vs. rituximab
(even with known CD20 negativity); and are inclined toward rituximab
therapy.  Given his underlying uncharacterized cellular immunodeficiency
the move to BMT is being entertained after treatment of the Castleman's.
I don't think he has an allogeneic match and would require MUD.  Flow
for SAP was normal. HIV testing has been negative.  I am wondering if
any of you have seen Castleman's in your immunodeficient patients and if
so what the treatment was and clinical course?  Thank you, Chris 

		On Jun 13, 2006, at 9:32 PM, K. Scott Baker wrote: 

			We have had 2 cases in the last couple of years,
the first ended up having XLP, the second we have been unable to
molecularly characterize a defect, he has a "CVID" phenotype.
Interestingly his lymphoma was metastatic Hodgkin's at age 4, which
recurred. He is being treated for the recurrence and then going to
allo-HCT. The XLP had several recurrences of his NHL (mainly abdominal
primaries). He received non-myeloablative MSD BMT 2 yrs ago and has had
no evidence of his disease (or xlp) since. Are you planning on HCT for
your patient? 

			K. Scott Baker, MD, MS 

			Pediatric Blood and Marrow Transplant Program 

			University of Minnesota 

			420 Delaware St. SE, Mayo Bldg. Room D557 

			Mayo Mail Code 484 

			Minneapolis, MN 55455 

			612.625.4952 FAX 612.626.1434 

			baker084 at tc.umn.edu 

			From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Christine
Seroogy 
			Sent: Tuesday, June 13, 2006 1:51 PM 
			To: pagid at list.clinimmsoc.org 
			Cc: Kakumanu Sujani 
			Subject: [PAGID] lymphopenia 

			Dear Colleagues, 

			I would like to get your opinions about a 15y/o
boy was admitted to our hospital with a gastrointestinal lymphoma
(further characterization pending.) He was initially seen in the
immunology clinic here at 4 years of age because of severe primary VZV
infection. Evaluation at that time demonstrated
lymphopenia--predominately CD4--and poor mitogen response. His B cell
numbers and function is normal (measured by titers to various vaccine
antigens); he has been persistently hypergammaglobulinemic (IgG 1000s
and IgA 400s). NK cell numbers by CD16/56 slightly diminished. HIV
nonreactive. He developed a Burkitt's lymphoma around age 10 and was
successfully treated, he has had refractory sinus disease and recurrent
OM. No other infectious history or autoimmune phenomenon. No relevant
family history. I welcome any thoughts on how to pursue a molecular
diagnosis on this child or hearing if you have seen similar cases. Thank
you, Chris 

			Chris Seroogy, M.D. 
			Assistant Professor 
			Dept. of Pediatrics 
			Mail: H4/474 CSC, Mailstop 4108 
			Shipping: H4/431 CSC, Mailstop 4108 
			600 Highland Ave. 
			Madison, WI 53792 
			phone: 608- 263-2652 
			fax: 608-265-0164 

	Kathleen E. Sullivan MD PhD 
	Chief, Division of Allergy and Immunology 
	Associate Professor of Pediatrics 
	The Children's Hospital of Philadelphia 
	(p) 215-590-1697 
	(f) 215-590-3044 

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