[CIS PIDD] [cis-pidd] Mannose Binding Lectin Deficiency
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Thu Feb 9 12:07:20 EST 2017
Unfortunately she has never had a Chest CT at our hospital, so I am trying
to reach her about if its ever been done at outside hospitals. Otherwise I
will order one for her.
Not that I know of she was worked up for NTMB. I will have to dig further
and I plan on boosting her. I will update with results
Thank you everyone!!
pamella
On Thu, Feb 9, 2017 at 11:26 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:
> Curious what her chest CT shows. Has NTMB infection been excluded? Agree
> with assessing response to pneumovax if not done previously.
>
> I generally think MBL is a cofactor for infectious risk when coupled with
> other immunodeficiency conditions, however I’ve seen 1 patient who
> developed recurrent pneumonia and extensive bronchiectasis with
> undetectable MBL and no other findings after an exhaustive immunologic and
> pulmonary eval, including for PCD. So I’d “never say never”. Have
> treated that patient with IVIG for a few months in hopes of slowing his
> lung disease, though difficult to say if it’s had a significant impact.
>
> Best,
>
> Marc
>
> Marc Riedl, MD, MS
> Professor of Medicine
> Adult Primary Immunodeficiency Program
> Division of Rheumatology, Allergy & Immunology
> University of California, San Diego
>
> 8899 University Center Lane, Suite 230
> San Diego, CA 92122
> Tel 858.657.5350 <(858)%20657-5350> Fax 858.657.5375 <(858)%20657-5375>
>
>
>
> From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net>
> Date: Thursday, February 9, 2017 at 7:58 AM
> To: CIS-PIDD <cis-pidd at lyris.dundee.net>
>
> Subject: Re: [cis-pidd] Mannose Binding Lectin Deficiency
>
> Thank you everyone for all your help and input!
> I will update you as soon as I have more information!
>
> Thank you
> pamella
>
> On Thu, Feb 9, 2017 at 10:52 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
>>
>> I meant Primary Ciliary Dyskinesia, sorry.
>>
>>
>>
>> Kathleen A. Haines, M.D.
>>
>> Section Chief, Pediatric Immunology
>>
>> Section of Pediatric Rheumatology & Immunology
>>
>> Joseph M. Sanzari Children’s Hospital
>>
>>
>>
>>
>> 30 Prospect Ave., Hackensack, NJ 07601
>>
>> T: 551-996-5306 <(551)%20996-5306> | F: 201-996-9815 <(201)%20996-9815> |
>>
>> Kathleen. Haines at HackensackMeridian.org
>>
>> HackensackMeridianHealth.org <http://hackensackmeridianhealth.org/>
>>
>>
>>
>>
>>
>>
>>
>>
>>
>>
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>> *From:* CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
>> *Sent:* Thursday, February 09, 2017 7:48 AM
>> *To:* CIS-PIDD <cis-pidd at lyris.dundee.net>
>> *Subject:* RE: [cis-pidd] Mannose Binding Lectin Deficiency
>>
>>
>>
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>> I would establish whether there is an adequate response.
>> ------------------------------
>>
>> *From:* CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
>> *Sent:* Wednesday, February 08, 2017 11:16 PM
>> *To:* CIS-PIDD
>> *Subject:* Re: [cis-pidd] Mannose Binding Lectin Deficiency
>>
>> Good Evening, These levels are pre pneumococcal titers and I had counted
>> 14/23 as greater than 1.3 which is around 60 percent so I didn't think a
>> booster shot was indicated.
>>
>> Do you think I should boost her and recheck?
>>
>>
>>
>> Thank you
>>
>>
>>
>> Pam A
>>
>> Sent from my iPhone
>>
>>
>> On Feb 8, 2017, at 11:05 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
>> wrote:
>>
>> Assuming that these are post vaccination pneumococcal titers, there are
>> 9/23 of the pneumococcal serotypes which are less than 1.3, so it is less
>> than 70%, which would classify as SAD moderate phenotype. There is an
>> evidence based review regarding replacement Ig in the Working Group Report
>> to be published next month in JACI which includes all patients with SAD,
>> including the mild phenotype. Similar findings were in the Practice
>> Parameter.
>>
>> Regards,
>>
>> Tony Tichenor
>> ------------------------------
>>
>> *From:* CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
>> *Sent:* Wednesday, February 08, 2017 8:48 PM
>> *To:* CIS-PIDD
>> *Subject:* [cis-pidd] Mannose Binding Lectin Deficiency
>>
>> Good Afternoon,
>>
>>
>>
>> I have a patient who is 69 year old AAF with Pmhx of asthma, hymenoptera
>> allergy, allergic rhinitis, several antibiotic allergies including
>> penicillin and recurrent pneumonias (2 times ended up in ICU at an outside
>> hospital), and sinusitis and an abscess
>>
>> She is on a beta blocker for her HTN and is not very excited about
>> pursuing hymenoptera or PCN testing or shots.
>>
>> 9/23
>>
>> However, when I completed her immune workup
>>
>> HIV Negative
>>
>> IgG/A/M/E all normal
>>
>> Pneumococcal Protective
>>
>> Diptheria/Tetanus Protective
>>
>> CH50 normal
>>
>> Cell Markers: CD8 mildly low and CD 56/16 high and CD19 high
>>
>> And her Mannose Binding Lectin was practically 0
>>
>>
>>
>>
>>
>> Below I have included her detailed immune history and her immune lab
>> results.
>>
>> What do we currently have to offer these patients besides Abx or
>> prophylaxis? Is anyone’s lab providing those infusions of MBL and does it
>> work? Is there any additional workup that needs to be done? (I thought
>> about oxidative burst, but we couldn’t get it done that day because it was
>> a send out)
>>
>> Any help would be appreciated!
>>
>>
>>
>> Thank you
>>
>> Pamella Abghari
>>
>> MetroHealth System
>>
>> Cleveland, Ohio
>>
>>
>>
>>
>>
>> Component 1/26/2017
>> Pneumo Ab Type1 4.1
>> Serotype 2 2.0
>> Pneumo Ab Type3 <0.3
>> Pneumo Ab Type4 2.0
>> Pneumo Ab Type 5 2.9
>> Pneumo Ab Type8 2.1
>> Pneumo Ab Type9N 4.5
>> Pneumo Ab Typ12 0.6
>> Pneumo Ab Typ14 11.0
>> Serotype 17F 0.4
>> Pneumo Ab Type 19F 0.8
>> Serotype 20 4.2
>> Serotype 22F <0.3
>> Pneumo Ab Typ23 0.3
>> Pneumo Ab Type6B 5.9
>> Serotype 10A 2.2
>> Serotype 11A 2.0
>> Pneumo Ab Type7F 1.2
>> Serotype 15B 0.9
>> Pneumo Ab Type 18C 1.7
>> Serotype 19A 0.3
>> Pneumo Ab Type 9V 3.6
>> Serotype 33F 4.5
>>
>> WBC 5.7
>> RBC 5.30 (H)
>> Hemoglobin 12.8
>> Hematocrit, spun 41.1
>> MCV 78 (L)
>> MCH 24.2 (L)
>> MCHC 31.1 (L)
>> Platelet 149 (L)
>> RDW-CV% 14.5
>> MPV 11.5
>> Neutrophils 44.7
>> Lymphocytes 44.3 (H)
>> Monocytes 6.9
>> Eosinophil 3.4
>> Basophils 0.7
>> Neutrophil # 2.54
>> Lymph Absolute 2.51
>> Monocyte Absolute 0.39
>> Eosinophil Absolute 0.19
>> Basophil # 0.04
>>
>> CD3 % Lymphs 67
>> CD3 Absolute Count 1703
>> CD3/CD4 % Lymphs 54
>> CD3/CD4 Absolute Count 1361
>> CD3/CD8 % Lymphs 13
>> CD3/CD8 Absolute Count 323 (L)
>> CD4/CD8 Ratio 4.2 (H)
>> CD16/56 % Lymphs 13
>> CD16/56 Absolute Count 334 (H)
>> CD19 % Lymphs 18
>> CD19 Absolute Count 456 (H)
>>
>> Tetanus Antibody 3.20
>> Diphtheria Antibody 0.16
>>
>> CH50 Complement >60 (H)
>>
>> IgA, Quantitative 271
>> IgE, Serum 6.4
>> IgG, Quantitative 913
>> IgM, Quantitative 118
>>
>> HIV-1/2 Eia Ab Nonreactive
>>
>> Mannose Binding Protein <0.5 (L)
>>
>>
>>
>>
>>
>> Birth History: C/S FT Complications Denies
>> Mom have any miscarriages Maybe, before her
>>
>>
>>
>>
>> Additional History:
>> FTT Denies
>> Chronic Diarrhea Denies
>> Abnormal facies Denies
>> Cardiac Defects Denies
>> Developmental or functional delays Denies
>> Fractures Hit her foot to the wall and broke her toe, fell 5 years ago
>> and broke her ankle
>> Teeth came in at age <1 year old Any abnormalities (weird shape, had to
>> be pulled) Denies
>> What age did umbilical cord fall off Denies
>> Immunizations UTD
>> Asthma Yes
>>
>>
>>
>>
>>
>>
>>
>> Infection History:
>> Otitis Media: A lot as a child, Had tymp tubes as an adult.
>> Pneumonia Yes Treated with Abxs Yes Ever admitted for antibiotic
>> treatment Yes
>> Any complications : 4in her lifetime. Admitted for 2 of them with one in
>> the CCU
>> Sinusitis Yes but doesn't get abx treatment
>> Meningitis Denis
>> Abscess Drained an abscess in her fallopian tube eventually had
>> hysterectomy
>> Cellulitis Denies
>> Osteomyelitis Denies
>> Thrush Denies
>> Viral infections (per year) 1-2x a year
>>
>>
>>
>>
>> ICU Yes refer to above
>> Hospitalizations Yes refer to above
>>
>>
>>
>> Family Hx of PID or Autoimmune Dzs (HIV, CVID, SCID,CGD, LAD, Crohns, UC,
>> RA): Denies
>>
>>
>>
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