[PAGID] Fwd: Re: Malakoplakia

[Jack Bleesing]

For those interested, I have 2 PDFs (rejected due to size limitations).
J

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Jack J.H. Bleesing, M.D., Ph.D.
Cincinnati Children's Hospital Medical Center
Division of Hematology/Oncology
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)
Jack.Bleesing at CCHMC.org
http://www.cincinnatichildrens.org/immunodeficiencies/



>>> Jack Bleesing 2/27/2008 9:04 AM >>>


Dear Lily/Anita:

If you approach this from the unusual foamy histiocyte and a bit of imagination, the foamy histiocyte is found in non-Langerhan's cel histiocytic disorders, such as Erdheim-Chester disease and xanthogranuloma, and perhaps Rosai-Dorfman disease in which one can find histiocytes with emperipoiesis. The foamy histiocyte seem to express CD68 and CD163 and points towards a differentiation pathway of macrophages (based on CD206 and CD163 expression - see atttached); whether a primary consequence of disease or a secondary consequence of infection, I am not sure about.

We see evidence of (we think) inappropriate macrophage differentiation in MAS and HLH disorders, characterized by increased presence of CD163 in blood (joints) due to cleavage of CD163 from the macrophage/histiocyte.

Looking at shared features may give some options ( immunosuppressive therapy, cytokine-directed therapy, etc).

Regards,

JB


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Jack J.H. Bleesing, M.D., Ph.D.
Cincinnati Children's Hospital Medical Center
Division of Hematology/Oncology
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)
Jack.Bleesing at CCHMC.org 
http://www.cincinnatichildrens.org/immunodeficiencies/ 



>>> Anita Gewurz <agewurz at rush.edu> 2/26/2008 6:17:52 PM >>>

Dear Colleagues:

We have a 30-year-old African-American female patient who has been  
diagnosed with colonic and renal malakoplakia (variant spelling,  
malacoplakia).  This is a very rare disease, as noted below FYI*.   
Similarities to CGD and Whipple's disease (WD) have been noted and  
individual cases with overlap of CGD or WD and malakoplakia  
reported.  Our patient has normal PBMC phenotypes and in vitro  
responses to mitogen stimulation, and negative testing for CGD or HIV  
antibody.  She is not on corticosteroid therapy and does not have a  
known risk factor for malakoplakia.  Neither has she responded to  
extended treatment with levofloxacin.  Her condition is worsening,  
and the prognosis is death from obstruction or other complications.   
Our questions are do you have any advice for treating or testing this  
patient?

Thank you.

Sincerely,

Lily Hwang MD, A/I Fellow-in-Training
Anita Gewurz MD <agewurz at rush.edu>
Section of Allergy/Immunology
Department of Immunology/Microbiology
Rush University Medical Center
1725 W Harrison - 117
Chicago IL 60612
TEL (312) 942-6296
FAX (312) 563-2201


*According to the 2005 eMedicine.com review by Ralph McKiel MD,  
Department of Dermatology, Wayne State University http:// 
www.emedicine.com/derm/topic872.htm and other sources, malakoplakia  
is an uncommon chronic granulomatous disease of the skin or viscera  
characterized by "foamy histiocytes" - macrophages containing  
Michaelis-Guttman bodies.  The latter are believed to result from an  
acquired defect in macrophage bactericidal activity, as bacteria such  
as E. coli, have been cultured from lesions, and resolution following  
lengthy treatment with quinolone or sulfonamide antibiotics has  
occurred.  The main risk factor appears to be immunosuppression.   
There is positive association with lymphoma, rheumatoid arthritis,  
diabetes and chronic corticosteroid therapy or immunosuppression for  
organ transplantation, but not with HIV infection.