[CIS PIDD] [cis-pidd] Mannose Binding Lectin Deficiency
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Wed Feb 8 22:37:02 EST 2017
I would be interested in the answer to this too... I have several kids with
MBL deficiency of this level, mostly significant, frequent sinus infections
who either require prophylactic antibiotics or aggressive early treatment
of URIs to avoid the development of chronic sinusitis. One patient has
already had several sepsis episodes including ICU admissions, and I warn
all my patients of this possibility since it's been well described as a
risk.
The only data I have seen on MBL infusions has been in the context of
sepsis, and hasn't been helpful as far as I can tell. I'm not aware of data
looking at prophylactic use, but I would bet it's prohibitively expensive
(especially compared to amoxicillin or augmentin as needed).
Cheers
Bennett
Connecticut Childrens Medical Center,
Hartford, CT
On Wednesday, February 8, 2017, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:
> 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.
>
> 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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