[CIS PIDD] [cis-pidd] Mannose Binding Lectin Deficiency
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Thu Feb 9 09:44:31 EST 2017
IgE is curiously low for a patient with asthma and a history of all these allergies. I think that MBL deficiency can be a cofactor with mild antibody deficiencies for increased problems with recurrent infections. I am following a young man with XLA who developed Blastomyces pneumonia and we discovered that he also has undetectable MBL. Also, we have seen more than one person with recurrent candidiasis with very low MBL levels. Has she had bronchoscopy?
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
Cell: 205-999-7688
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, February 09, 2017 6:48 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: RE: [cis-pidd] Mannose Binding Lectin Deficiency
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<mailto: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<mailto: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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