[CIS PIDD] [cis-pidd] catastrophic antiphospholipid syndrome (CAPS)

Thu Dec 3 19:40:12 EST 2015

Certainly!

Normal CBC's.; WBC count 9.51 at the time of subsets

Lymph subsets (11/17, post several ritux treatments):
CD3 – 2,445
CD4 – 1,077
CD8 – 1,276
NK – 24
CD19 – 0; (no previous B cell panels available)

(This week, shortly post first bortezomib)
IgG: 615
IgM: 58
IgA: 90

 (Prior to bortez)
B2 microglobulin IgG - <9
B2 micro IgM - 100
B2 micro IgA - <9

Anti-cardiolipin IgA <11
Anti-cardio IgG <14
Anti cardio IgM 20

Lupus anticoagulant not detected (previously high titer positive)

Myeloperoxidase Ab 4.2
Anti PR3 Ab <1.0

Phosphatidylserine:
IgG <10
IgM >100
IgA <20

Since eculizumab started, all values:
Total Complement <10
CH50 <10

ANA, ANCA negative (post pheresis)

Blachy J. Dávila Saldaña, MD
Attending Physician, Division of Blood and Marrow Transplantation
Children's National Health System
Assistant Professor of Pediatrics
George Washington University School of Medicine and Health Sciences
111 Michigan Avenue, N.W.
Washington, DC 20010
Phone: 202-476-4561
Fax: 202-476-2280

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, December 03, 2015 9:00 AM
To: CIS-PIDD
Subject: RE:[cis-pidd] catastrophic antiphospholipid syndrome (CAPS)

Can you provide more details on his immunologic profile – flow cytometry, immunoglobulin levels, complement tests…

Prescott

T. Prescott Atkinson, MD PhD, Professor and Director
Division of Pediatric Allergy, Asthma & Immunology
University of Alabama at Birmingham
Tel: 205-996-9582
Fax: 205-975-7080

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Wednesday, December 02, 2015 10:28 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: [cis-pidd] catastrophic antiphospholipid syndrome (CAPS)

Hello all;

   The hematology team is dealing with a very complex patient I would like to hear your opinions on.  He is a 19 year old with CAPS who has had a very complicated course.  Below is a summary done by his primary hematologist.

  He presented in June 2012(age 17)  with a left sided DVT and PE after a flight to Denver.  He was triple positive for lupus anticoagulant, beta 2 glycoprotein I and anticardiolipin antibodies – high levels (>150).  He started anticoagulation and stabilized.  We tried catheter directed thrombolysis with limited success and placed a stent in the left iliac vein due to May Thurner Syndrome.  This stent thrombosed and he has many lower extremity collaterals with persistent clot.  He had several recurrent thrombosis on Coumadin and Rivaroxaban.  Only Lovenox seems to hold him.  Over the past year, he developed hemoptysis and has had several admissions with hemoptysis and recurrent pulmonary emboli.  He has a thrombus in his IVC infrarenal.  Rheumatology diagnosed him with MPO+/ANCA+ vasculitis and started steroids and monthly cyclophosphamide IV.  He was also treated with Rituximab 750/m2 x2.  He stabilized through the summer but then had recurrence x 2.  At the last hospitalization in September, he was treated for CAPS with plasmapharesis x 5 days, corticosteroids and IVIG.  Second course of Rituximab started x 2 doses.  He improved and prior to discharge they started eculizumab to try to reduce recurrence risk.  We continued eculizumab weekly and weekly pulse methylprednisone.  Readmitted with hemoptysis to CNMC; imaging showed recurrent pulmonary embolism.  Plasmapharesis x 5 days again.  Only thing that seems to help.  B2GPI and ACLA were normal post plasmapharesis and one week later ACLA 80 and B2GPI >150 (both IGM); B cell number 0.  We have decided to add Bortezomib to reduce IgM and plasma cells.  He is going to get Plasmapharesis/bortezomib on day 1 (11/30), day 4, day 8 and day 11.  After this, plasmapharesis will go weekly and will  have to decide on Bortezomib plan.

  He had been well prior to age 17; no dysmorphic features or known endocrinopathies.  Eculizumab seemed to help at least temporarily; I think the pheresis/bortezomib makes good theoretical sense.  Any further thoughts on treatments?  Or any further diagnostics?  Additionally, hematology has asked about transplantation for this patient. I’ve only seen a couple adult case reports of auto-BMT in the literature; none in the pediatric age.  He is not a good candidate, given his multiple PEs and tenuous respiratory status, but I wanted to know if there is more experience on this.

Thanks in advance.

Blachy

Blachy J. Dávila Saldaña, MD
Attending Physician, Division of Blood and Marrow Transplantation
Children's National Health System
Assistant Professor of Pediatrics
George Washington University School of Medicine and Health Sciences
111 Michigan Avenue, N.W.
Washington, DC 20010
Phone: 202-476-4561
Fax: 202-476-2280

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