[PAGID] Autoimmune Cytopenia in a 4 month old

[Jason W. Caldwell]

Yes DAT was positive before IVIG. Sorry that was not clear.  There were
no signs of erythopahocytosis and I made a point of asking.  He also has
no other signs or markers of HLH.  To be honest Heme/Onc is as asking me
what else to do at this time.  They are concerned about rituxin before a
diagnosis.   Thank you for the input.

 

Jason W. Caldwell DO

Assistant Professor of Internal Medicine & Pediatrics

Section Pulmonary, Critical Care, Allergy, and Immunological Diseases

Wake Forest University School of Medicine

Medical Center Boulevard

Winston-Salem, North Carolina, 27157

Office Phone: 336-716-5166

Fax:  336-716-7277

E-mail:  jcaldwel at wfubmc.edu

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Berger, Melvin
Sent: Thursday, September 16, 2010 12:14 AM
To: pagid at list.clinimmsoc.org
Subject: RE: [PAGID] Autoimmune Cytopenia in a 4 month old

 

Do you know if DAT was positive before IVIG ?  Are there clusters of
lymphocytes in marrow ?

 

 If it was just auto-antibody mediated peripheral destruction of all
formed elements of blood, marrow would show hypercellularity affecting
all lineages, so hypocellularity, "dyseryrthopoesis" and myeloid
hypoplasia might be more in favor of a viral suppression- parvo B19
usually affects erythropoesis more than others, so does not seem very
likely. If it does seem antibody mediated, you might try rituxin sooner
rather than later, but bear in mind that IVIG can cause Coombs'
positivity. Does marrow show erytrophagocytosis ?

 

Melvin Berger, M.D., Ph.D.

Adjunct Professor of Pediatrics and Pathology

Case Western Reserve University

Cleveland, OH 44106

 

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Jason W. Caldwell
Sent: Wed 9/15/2010 4:44 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] Autoimmune Cytopenia in a 4 month old

I have a patient that I would like the groups input.

This is a now 5 month old male that was admitted Wake Forest on Aug. 24.
He was four months old at the time of admission and had previously been
health with no known pregnancy or birth complications.  Mom is health
except for untreated depression.  No known meds during pregnancy.  Two
weeks prior to his admission he had a 10 day treatment for a "sinus
infection" with Augmentin.  He finished this 4 days prior to admission.
On the day of admission noted to have fever of 104 and pale.  Went to
outside ER and found to have a WBC of 3.8,  Hb of 6.2, Plt 3000, MPV of
18.9(6.8-10.2).  ANC of 200.  Original CMP was unremarkable including
normal ALT and AST.  Uric acid 4.0(2-8).  LDH 234(200-700). Ferritin
89(30-300).  DAT broad spectrum positive.  Reticulocytes peaked at 17.9
%(.2-2.5%).HIV negative by DNA and serology.  EBV PCR <300 copies.
Blood and urine culture are negative.

Immunology:  IgA 40 mg/dl; IgM 420 mg/dl;  IgG 1013 mg/dl (DONE AFTER
TREATMENT with IVIG);  I did not ask for titers since IgG had been given
before I was involved.  Lymphocytes 6700, CD19 1,530 or 22%;  CD3 4960
or 71%;  CD4 2900 or 43%; CD8 1880 or 28%; ratio 1.5; NK 70 or 15.  The
lab here reported CD3+, CD4-, CD8- at 3.1%, but a repeat sent to another
lab was 1.5%.  An LKP panel was sent and is not supportive of XLP.  NK
function, Response to mitogens, and SAP were seen but are all pending.

ROS/PE: other than being pale, he looks great, smiling, is eating,
urinating, and stooling, no respiratory symptoms or findings, no
abdominal findings or symptoms, NO adenopathy, spleen tip is palpable,
but liver is not, no rash or any skin findings.

Chest film is normal.

Bone marrow was normocellular with prominent myeloid hypoplasia,
dyserythroposiesis.

The above and 2 dose of IVIG at 1g/kg and high dose steroids were done
before I was consulted.  Despite this treatment he is still requiring
platelets and red blood cells ANC did rise briefly after steroids were
started, but is now back to 200.  Hb today of 8 (PTF) and platelet of 4.

  I had suggested extending the viral investigations with CMV,
Adenovirus, HHV-6, not yet done.  I have a concern for ALPS and have
recommended the molecular analysis be sent.  The team sent XLP molecular
analysis.  I suggested expanding the differential to IPEX and send for
FoxP3 expression.

Despite not having many of the clinical manifestations of many of the
immune deficiencies with autoimmunity, but him being so young and having
a rather severe autoimmune disorder:

Should I be considering APECED, Hyper IgM?

Is there any suggestion for other infectious causes?

When would you consider alternative treatments, despite not having a
definitive diagnosis?

I thank you in advance for support, agreement or criticism.

 

 

Jason W. Caldwell DO

Assistant Professor of Internal Medicine & Pediatrics

Section Pulmonary, Critical Care, Allergy, and Immunological Diseases

Wake Forest University School of Medicine

Medical Center Boulevard

Winston-Salem, North Carolina, 27157

Office Phone: 336-716-1250

Fax:  336-716-7277

E-mail:  jcaldwel at wfubmc.edu

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