[CIS-PAGID] nontypeable haemophilus influenzae and panniculitis in 14 day old female

[Patsy Giclas]

Did you rule out a complement defect?  How about a CH50? AH50?

Patsy
 
---------------------------------------------------------
Patricia C. Giclas. Ph.D.
Director, Complement Laboratory
Advanced Diagnostic Laboratories
Professor, Pediatrics Dept,  Allergy and Immunology Division
National Jewish Health
1400 Jackson St., Denver, CO 80206 U.S.A.

Office: D409, Neustadt Building
Phone: 303-398-1217
Fax: 303-270-2128
Email: giclasp at njhealth.org



> From: "Heimall, Jennifer" <heimallj at email.chop.edu>

> Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Date: Mon, 26 Sep 2011 22:10:08 -0600

> To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Subject: Re: [CIS-PAGID] nontypeable haemophilus influenzae and panniculitis

> in 14 day old female

> 

> Hi Allyson,

> 

> Did she have lymphocyte mitogens done, TRECs and/or memory markers on the T

> cells?  

> Her original alc was pretty low and her cd8 count is slightly lower than the

> normal range, though that could be from her acute illness. I'd want to rule

> out an atypical SCID.

> 

> Jen Heimall  

> Sent from my Verizon Wireless Phone

> 

> 

> -----Original message-----

>> From: "Larkin, Allyson" <allyson.larkin at chp.edu>

>> To: "<pagid at list.clinimmsoc.org>" <pagid at list.clinimmsoc.org>

>> Sent: Tue, Sep 27, 2011 00:01:40 GMT+00:00

>> Subject: Re: [CIS-PAGID] nontypeable haemophilus influenzae and panniculitis

>> in 14 day old female

>> 

>> Thank you. Tmax was 38.9. I don't have ESR/CRP. TLR assay pending.

>> Allyson

>> 

>> On Sep 26, 2011, at 7:50 PM, "Jyonouchi, Soma C"  wrote:

>> 

>>>> Does the patient mount an inflammatory response (fever, crp)?  This

>>> clinical picture can be seen in TLR defects (IRAK4, MyD88).  A TLR assay

>>> would be a useful screen.

>>>> 

>>>> Soma Jyonouchi, MD

>>>> Children's Hospital of Philadelphia

>>>> Division of Allergy and Immunology

>>>> ________________________________________

>>>> From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org]

>>> On Behalf Of Larkin, Allyson [allyson.larkin at chp.edu]

>>>> Sent: Monday, September 26, 2011 5:46 PM

>>>> To: pagid at list.clinimmsoc.org

>>>> Subject: [CIS-PAGID] nontypeable haemophilus influenzae and panniculitis in

>>> 14 day old female

>>>> 

>>>> Dear Colleagues,

>>>> 

>>>> I am requesting help regarding a 14 day old female with nontypeable

>>> haemophilus influenzae bacteremia, meningitis and skin lesions that show

>>> acute neutrophil rich necrotizing panniculitis.

>>>> 

>>>> 

>>>> 

>>>> History: First baby to non cansanguinous parents. Full term pregnancy.

>>> Mother on methadone. Forceps delivery for fetal  bradycardia. Low Apgars at

>>> birth. Went to NICU with neutropenia, tachypnea, abdominal distension and

>>> poor feeding. Blood cultures grew nontypeable H.Flu. CSF consistent with

>>> meningitis.  Clinical status improved with antibiotics, off vent, feeding

>>> but 

>>> infant developed large, soft masses on neck and lower back (2.1x0.9x2.4cm

>>> and 

>>> 1.7x0.7x2.6 cm). Bx showed neutrophil rich necrotizing panniculitis. Drained

>>> but sterile. Umbilical cord has separated with no complications.

>>>> 

>>>> 

>>>> 

>>>> Labs/Studies:

>>>> 

>>>> ·        Admission CBC: WBC 2.1, Hgb 14, Hct 39, Plts 39, abs neuts 1030,

>>> abs lymphs 420 (she than had multiple plt transfusions)

>>>> 

>>>> ·        Most recent CBC: WBC 14, Hgb 13, Hct 38.5, plts 255, Abs neuts

>>> 6920, abs lymphs 4750

>>>> 

>>>> ·        IgA 22, IgG 625, IgM 135

>>>> 

>>>> ·        HIV antibody screen negative

>>>> 

>>>> ·        Skin Bx: extensive necrosis of the panniculus with diffuse

>>> neutrophilicinfiltrate.  The keratinizing squamous epithelium is does not

>>> show parakeratosis, acanthosis, or spongiosis.  The superficial dermis and

>>> adnexalstructures are largely uninvolved. The deeper reticular dermis shows

>>> marginating neutrophils with a neutrophilic exudate around the vessels with

>>> a 

>>> diffuse neutrophilic infiltrate in the subcutaneous tissue.  This

>>> neutrophil-rich inflammatory infiltrate has associated basophilic necrosis.

>>> The sheets of neutrophils and necrosis are the predominant feature with no

>>> restriction of the inflammatory process to any compartment of the

>>> panniculus. 

>>> Macrophages are not a predominant feature. No significant crystallization of

>>> the fat. There is no evidence of immature leukemic blasts. Gram stain

>>> negative. Grocott and PAS are negative for fungi. Fite stain is negative for

>>> Nocardia.

>>>> 

>>>> ·        NOBA: Peripheral blood was evaluated for neutrophil oxidative

>>> burst activity in the presence and absence of phorbol myristate acetate

>>> (PMA), a stimulator of protein kinase C. This study demonstrates neutrophil

>>> oxidative burst activity with a normal stimulation index, but also shows

>>> slight pre-activation of the cells. These findings do NOT suggest chronic

>>> granulomatous disease.

>>>> 

>>>> ·        Subsets:

>>>> 

>>>>    % T-Cells (CD3) 61 ( Ref. Range 58 - 64)

>>>>                                            Total T-Cells (CD3) 3,400 (Ref.

>>> Range 2,400 - 3,300)

>>>>                                            % Helper Cells (CD4) 49 (Ref.

>>> Range 36 - 50)

>>>>                                            Total Helper Cells (CD4) 2,761

>>> (Ref. Range 1,600 - 2,200)

>>>>                                            % Suppressor Cells (CD8) 11

>>> (Ref. Range 20 - 30)

>>>>                                            Total Suppressor Cells (CD8) 635

>>> (Ref. Range 820 - 1,600)

>>>>                                            % B-Cells (CD19) 28 (Ref. Range

>>> 22 - 29)

>>>>                                            Total B-Cells (CD19) 1,593 (Ref.

>>> Range 1,000 - 1,600)

>>>>                                            % NK Cells (CD16/CD56) 7 (Ref.

>>> Range 7 - 21)

>>>>                                            Total NK Cells (CD16/CD56) n 372

>>> (Ref. Range 270 - 1,100)

>>>>                                          Helper/Suppressor Ratio 4.35 (Ref.

>>> Range 1.70 - 3.10)

>>>> 

>>>>  *   Abdominal ultrasound: normal liver, spleen

>>>> Assessment:

>>>> 

>>>>  *   I am concerned about a phagocytic disorder. I am thinking of

>>> neutrophil specific granule deficiency. Her peripheral blood smear looked

>>> suspicious to me for bilobed nuclei in the neutrophils (awaiting heme path

>>> review).

>>>>  *   I was hoping there might be suggestions/thoughts about other specific

>>> primary immunodeficiencies that might fit her clinical description.

>>> Additionally, I was wondering if anyone had suggestions about further

>>> workup:

>>>> 1) Performing electron microscopy to look at number of specific granules

>>>> 2) Look at the granule proteins by Elisa

>>>> 3) Look for mutation in CEBPE

>>>> 4) Is there a role for bone marrow biopsy?

>>>> 5) Is there a role for prophylactic antibiotics during this workup?

>>>> 

>>>> Thank you kindly for your thoughts,

>>>> 

>>>> Allyson Larkin, MD

>>>> Assistant Professor of Pediatrics

>>>> Department of Pulmonary Medicine, Allergy and Immunology

>>>> Children¹s Hospital of Pittsburgh

>>>> One Children¹s Hospital Drive

>>>> 4402 Penn Avenue

>>>> Pittsburgh, PA 15224

>>>> 

>>>> Phone: 412-692-8903

>>>> Fax: 412-692-8499

>>>> E-mail: allyson.larkin at chp.edu




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