Wednesday, June 22, 2011

A patient with leprosy who is awaiting renal transplant in the USA

Leprosy Mailing List – April 6th, 2011 
Ref:     A patient with leprosy who is awaiting renal transplant in the USA
From: Laura O. Coster, George Town University, USA

Dear Dr. Noto,
Dr Barbara Stryjewska from the Hansen's Institution recommended that I ask for your insight on a patient who is being evaluated at Georgetown University who was seen for pre-transplant Infectious Disease evaluation.  His previous medical care had been at an outside Hospital and Leprosy care through an outside Infectious Disease MD.  It is a very complicated case and I don't expect you to answer the below questions but if you could share your experience it would be very appreciated.
The major questions which are under debate:
1) Is his renal disease secondary to Leprosy;
2) Was Cryoglobulinemia responsible for his Renal disease;
3) Is there any contraindication to Renal Transplant;
4) Given his past history of Cytoxan (Cyclophosphamide. Also know as Endoxan) therapy which likely exacerbated his leprosy and substandard leprosy therapy how long should he be treated for Leprosy with traditional 3 drug therapy prior to transplant?
His story:
This 35 yr old man came from Bolivia in 2001 and was not diagnosed with Leprosy until 2007.  He developed endstage renal disease after 1 yr of Leprosy treatment in 2008, in detail:
- 2000: Diagnosed with "rheumatic fever" in Bolivia (the patient could not provide any details).  According to notes (but patient denies) he had recurrent pharyngitis from 2000- 2006.     
- 2001: Leg edema and elevated creatinine in the US.  Renal biopsy: Membrano-proliferative Glomerular Nephritis with crescent formation.  Significant mesangial capillary proliferation. 40% Crescent formation.  Staining of glomerular capillary walls and mesangium for IgG, C1a kappa and lamda.  Differential: cryoglobulinemia vs immunotactoid glomerulonephropathy vs. SLE.  At this time of this biopsy Cryoglobulins were negative and the diagnosis of Immunotactoid glomerulonephropathy was favored.  However, the staining pattern of both kappa and lambda light chain staining was atypical for Immunotactoid Glomerulonephritis.  Hand and arm warts were "diagnosed" at this time.
- December 2005: He presented with leg and facial swelling.  As per notes he had progressive neuropathy, splenomegaly, positive Cryoglobulins in blood (IgG kappa and IgM lambda) and his Creatinine was 2.1.  He was treated for "Essential mixed Cryoglobulinemia" with Cytoxan and Prednisone at least for a month.
- February 2006: Hospitalized for fever, tonsillitis, necrotic vasculitis of L ear and hands, new nephrotic syndrome and lower extremity.  At this point Cytoxan was stopped and patient was treated with plasmapheresis with good response.
Renal biopsy: "Immune complex-mediated glomerulonephritis with memrano-proliferative and focal crescentic patterns, most likely associated with Cryoglobulinemia".  At this hospitalization the patient had Cryoglobulins measurable in blood for some time.
Also patient had tonsillectomy which was read at the time as "actinomycetes structures".  (We recently sent the tonsillar biopsy to the Hansen's institute and it is actually c/w Lepromatous Leprosy).
- September 2007: Patient presented with nose bleed and a nasal biopsy and hand biopsy at this point were read as with Lepromatous Leprosy.
- September 2007/2008: Patient was initially treated with Dapsone/Rifampin for leprosy.  Because of non-hemolytic anemia on regimen he was changed to Minocycline/Rifampin x 12 months.  For unclear reasons the patient never received Clofazamine containing regimen.  During treatment for Leprosy his Creatinine increased from 2.1 to endstage renal disease by October 2008.
- February 2009: Patient was being evaluated for renal transplant so Hansen's Institute was involved.  A  forearm biopsy was compared to previous biopsy read Lepromatous Leprosy (LL-BL) in regression.  The Hansen's Institue recommended longer length of therapy given plans for renal transplant.  Again, for unclear reasons the patient was treated with non-Clofazamine regimen of Dapsone 25 mg qd/Rifampin monthly x 1 year.
- August 2010:  Skin biopsy of ear "Bacillary load within inflammatory area is decreased as compared to 04/09 arm biopsy. His first exam at Georgetown notable for Thinning eye brows, L ear pinna thinned, scattered macules on arm Hypertrophy on hand skin, bulla on palmar surface of distal digits, ballooning of digits. Decreased sensory sensation on face, arms (from biceps down) on legs (from thigh down). Multiple burn scars on legs.  He has a Peritoneal Dialysis catheter.
- January 2011: There was a National Teleconference through the Hansen's Institute where patient was treated  with the consensus that the patient should be treated at least 1 more year as he had substandard therapy.  The consensus was also that there was no contraindication to transplant as patients with leprosy did well if they were followed closely and new lesions were  treated.  At Georgetown, we started him on Dapsone, monthly Rifampin and Clofazamine in preparation for transplant. Unfortunately, he had non-hemolytic anemia again on Dapsone (he had not taken Rifampin yet) and will be changed to Minocycyline/Rifampin and Clofazamine.  It is thought that Cytoxan treatment in 12/05 increased the bacillary load and his 02/06 hospitalization with vasculitis could have been an ENL reaction.
Thank You for any thoughts on this difficult case, Laura
Laura O. Coster, M.D.
Assistant Professor of Medicine
Division of Infectious Diseases and Travel Medicine
Georgetown University Hospital
(202) 444-0153 (phone)
1-877-665-8072 (fax)

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