[CIS PIDD] [cis-pidd] 45 yo man with immunodeficiency and recurrent basal cell and squamous cell carcinoma

[Richard Wasserman]

I am posting this case in behalf of my colleague.


45yo male presents for immune evaluation due to recurrent basal and
squamous cell carcinoma and immunodeficiency.

The patient reports a long-standing history of recurrent sinopulmonary
infections since childhood. He has undergone bilateral myringotomy tube
placement several times and adenoidectomy. He has been treated for
bacterial pneumonia ~6 times in his lifetime.

There has also been a recurrent problem with warts (perianal, genital and
hands). He reports WHIM genetic testing (I assume CXCR4 Gene Sequencing)
has been negative in the past.

He also reports a severe outbreak of chicken pox as a child, requiring
hospitalization for 10 days. It was complicated by viral meningitis. As an
adult, he has had several cases of pneumonia of presumed viral etiology.

There is a prior history of specific IgM deficiency and he was previously
treated with IGIV due to a poor response to Pneumovax. His last infusion
was  about 9 month ago. In those 9 months, he reports being treated for
9-10 sinus infections. Prior allergy testing to common aeroallergens was
negative.

Patient underwent balloon sinuplasty in 2008. Biopsy of his nares at that
time showed a basal cell carcinoma. One year later, he had a spindle cell
carcinoma on his left ear requiring excision and grafting. Since then, he
has had 2-3 basal cell carcinoma removed, and about 12 biopsy-proven
squamous cell carcinomas.

Three months prior to presentation, he underwent removal of a squamous cell
carcinoma and grafting of his left cheek. He also required chemotherapy
(cetuximab, carboplatin, paclitaxel). He is currently weaning off of
prednisone as well.

His most recent laboratory evaluation is below:

Strep pneumo titers were <0.3 mcg/mL to 23 serotypes. Post-Pneumovax titers
pending.


Total Memory B-cell % Abs CD19+/CD27               25%
9-64

Total Memory B-cell Absolute CD19+/CD27+        12(L)cells/uL
  18-242

Class-switched Memory % CD19+/CD27+/IgD-     11%
   4-40

Class-switched Absolute CD19+/CD27+/IgD-          5(L)cells/uL
7-155

Non-switched Memory % CD19+/CD27+/IgD+       14%
3-35

Non-switched Absolute CD19+/CD27+/IgD+            7cells/uL
5-100

Naive B-cell % CD19+/CD27-/IgD+                           65%
0-100

Naive B-cell Absolute CD19+/CD27-/IgD+               30cells/uL
5-345

B-cells % CD19
  2(L)%                        6-28

B-cells Absolute CD19
46(L)cells/uL            94-588


PHA, Cpm                   L 25214 (Net CPM)
  73700-265000


Con A, Cpm                L 14915 (Net CPM)
 46100-283000
EZ

PWM, Cpm                 L 11473 (Net CPM)
29,100-125,000



Immunoglobulin A              161 (mg/dL)                           81-463
mg/dL

Immunoglobulin G           L 470 (mg/dL)                        694-1618
mg/dL

Immunoglobulin M             L <5 (mg/dL)                           48-271
mg/dL

Immunoglobulin E                   3 (kU/L)                         <OR=114
kU/L



Tetanus Toxoid Antibody   0.58 (IU/mL)

Diphtheria Antitoxoid Ab   0.08 (IU/mL)



Any further recommendations for additional laboratory evaluation?

Besides resuming immunoglobulin replacement therapy, any other
recommendations for therapy?


Thank you.


Richard Wasserman

Dallas
-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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