[CIS PIDD] [cis-pidd] VS: 17 yr old WF with CVID and neutropenia

[Seppänen Mikko]

Agree with earlier responses, however, relevant for this patient's present and future therapies, I would also like to suggest you read 2 recent and in my mind excellent articles:

Splenectomy (which at times may be needed, this is a bit comforting):

Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients.
Wong GK, Goldacker S, Winterhalter C, Grimbacher B, Chapel H, Lucas M, Alecsandru D, McEwen D, Quinti I, Martini H, Schmidt RE, Ernst D, Espanol T, Vidaller A, Carbone J, Fernandez-Cruz E, Lougaris V, Plebani A, Kutukculer N, Gonzalez-Granado LI, Contreras R, Kiani-Alikhan S, Ibrahim MA, Litzman J, Jones A, Gaspar HB, Hammarstrom L, Baumann U, Warnatz K, Huissoon AP; Clinical Working Party of the European Society for Immunodeficiencies (ESID).
Clin Exp Immunol. 2013 Apr;172(1):63-72. doi: 10.1111/cei.12039.
(being a child and OPSI w/o prophylaxis may be a very real threat, if one absolutely needs to splenectomize, prophylaxis definitely indicated)

Granulomatous lymphocytic lung disease (small, groundbreaking, unavoidably open study w/o control group):

Use of combination chemotherapy for treatment of granulomatous and lymphocytic interstitial lung disease (GLILD) in patients with common variable immunodeficiency (CVID).
Chase NM, Verbsky JW, Hintermeyer MK, Waukau JK, Tomita-Mitchell A, Casper JT, Singh S, Shahir KS, Tisol WB, Nugent ML, Rao RN, Mackinnon AC, Goodman LR, Simpson PM, Routes JM.
J Clin Immunol. 2013 Jan;33(1):30-9. doi: 10.1007/s10875-012-9755-3. Epub 2012 Aug 29.

mikko

Mikko Seppänen, MD, PhD
Immunodeficiency Unit
Helsinki, Finland

________________________________
Lähettäjä: Akhter, Javeed [mailto:javeed.akhter at advocatehealth.com]
Lähetetty: 29. toukokuuta 2013 20:31
Vastaanottaja: CIS-PIDD
Aihe: [cis-pidd] 17 yr old WF with CVID and neutropenia

Hi friends
I need your input with a 17 yrwf with CVID diagnosed approximately 10 yrs ago. Here is a brief summary.

·        Her presentation was of Evan's syndrome early in life

·        At 7 or 8 yrs of age she had a LLL pneumonia and on imaging multiple small nodules in the lung parenchyma as well as mediastinal lymphadenopathy.  Both the mediastinal nodes and the pulmonary nodules revealed non-caseating granulomas.  This is when I saw her and diagnosed CVID (probable according to the ESID criteria)

·        She was also noted to have splenomegaly that has increased in size and is currently 17.5 cm linear measurement on ultrasound

·        She was started initially on IVIG and now SCIG weekly with an additional dose of IVIG every 2-3 months to keep the IgG lvels high (empiric therapy following personal communications with dr Charlotte Cunningham-Rundles).  She was also started on po steroids initially daily and currently qod.  She is down to 10 mg qod

·        At around 12 years of age her thrombocytopenia became severe and nonresponsive to increased steroids therefore she was given four doses of Riuximab.  She responded well to it.

·        With IG replacement and steroids and possibly Rituximab she has done well including with her lung disease that is almost completely resolved

·        Last week she came in with a viral syndrome, low grade fever, aches and pains and severe neutropenia with an absolute count of 100-200.  She was given three doses of Nuprogen with no response.  This is the first time she has had severe neutropenia.  NP swab for viral PCR is negative.

·        There is no thrombocytopenia or anemia.  The lung function and xray are fine. No other new physical findings.  She looks healthy
What should be my next step
? bone marrow biopsy
? more Nuprogen
? blast of steroids, oral or pulse
? and finally what is your opinion on splenectomy on this young woman

Thank you much

Javeed Akhter, M.D.
Section Head
Pediatric Pulmonology
Allergy and Immunology
Director
Cystic Fibrosis Care Center
JMF Immunology Referal Center
p 708.684.5810
F 708.684.2045


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