Beruflich Dokumente
Kultur Dokumente
Rojas
Historia
Primera descripcin por Paul Kaznelson en 1922
Caso clnico inicial:
Paciente masculino de 58 aos de edad, con anemia progresiva,
cuenta eritrocitaria de 0.55x1012, con leucocitos y plaquetas
normales.
La autopsia mostr total ausencia de progenitores eritroides con
adecuada maduracin en la lnea de granulocitos y megacariocitos.
Yataro Yoshida. Historial review. The light and shadow of Paul Kaznelson: his life and
contribution to hematology. Ann Hematology (2008) 87:877-87
Definicin
Ronald Hoffman MD,Edward J. Benz Jr. MD,Leslie E. Silberstein MD. Hematology: Basic Principles and Practice, 6e
Clasificacin
Congnitas
Diamond- Blackfan
Autoinmunes
Idiopticas
Ronald Hoffman MD,Edward J. Benz Jr. MD,Leslie E. Silberstein MD. Hematology: Basic Principles and Practice, 6e
Incomp.
ABO
Hematolgic
as
LLGG,SMD,LNH
MM,LLA
Farmacolgica
s
Secundari
as
Autoinmun
es
Tumores
Solidos
Infeccione
s
Tiroides, Renal
Adenocarcino
ma
Timoma
Ronald Hoffman MD,Edward J. Benz Jr. MD,Leslie E. Silberstein MD. Hematology: Basic Principles and Practice, 6e
Frmacos
David M. Mintzer, Shira N. Billet, and Lauren Chmielewski .Drug induce hematologuc Syndromes . Adv. In hematology
Embarazo
FR:
Medroxiprogesterona
Choudry MA1, Moffett BK, Laber DA.Pure red-cell aplasia secondary to pregnancy, characterization of a syndrome.Ann
Hematol. 2007 Apr;86(4):233-7. Epub 2007 Jan 30.
PATOFISIOLOGA
En la mayora de los casos el
evento inicial no se conoce
Anticuerpos especficos
en contra de
eritroblastos
Citotoxicidad directa de los
linfocitos T (CTL)
Hedi Orbach, Gisele Zandman Goddard, Asher Winder and Yehuda Shoenfeld. Acquired adult pure red cell aplasia. Diagnostic criteria in autoinmune disease.
2008
C3b
Precurs
Precurs
or
or
eritroide
eritroide
Citotoxicidad directa por
linfocitos T
Policlonal
Mielodisplasia
APCR
Wang SA, Yue G, Hutchinson L, et al. Myelodysplastic syndrome with pure red cell aplasia shows characteristic clinicopathological features and
clonal T-cell expansion. Br J Haematol 2007; 138:271.
Timoma
Bone marrow biopsy of a patient with pure red cell aplasia (A) showing
an abundance of myeloid cells and some megakaryocytes. Erythroid
precursors are absent. Contrast with normal marrow of another patient
(B) showing numerous erythroid precursors (cells with dark, round
nuclei; a cluster is seen within the encircled area) (hematoxylin and eosin,
40).
Se presenta en
aproximadamente 5%
de los pacientes con
APCR
Puede coexistir con
miastenia gravis
Thompson CA, Steensma DP. Pure red cell aplasia associated with thymoma: clinical insights from a 50-year single-institution
Parvovirus b19
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Brown KE1, Anderson SM, Young NS.Erythrocyte P antigen: cellular receptor for B19 parvovirus. Science. 1993 Oct 1;262(5130):114-7.
Receptor
de
antgeno P
Integrina 51
Los glbulos rojos humanos maduros, que expresan altos niveles de receptor de antgeno P , permiten la unin del virus pero no la entrada viral porque
carecen de la integrina 51 correceptor (A), mientras que las clulas progenitoras eritroides , que expresan tanto receptor de antgeno P y correceptor
integrina 51 , son permisivas para parvovirus B19 entrada (B )
Kirsten A. Weigel-Kelley, Mervin C. Yoder, and Arun Srivastava. 51 integrin as a cellular coreceptor for human parvovirus B19: requirement of functional activation of 1 integrin for viral entry.
December 1, 2003; Blood: 102 (12)
Exposicin reciente:
Caractersticas
morfolgicas:
Proeritroblastos gigantes
Serologa
Anticuerpos IgM
Anti-B19
Confirmacin
DNA B19
Uptodate 2014
Frickhofen N, Chen ZJ, Young NS, et al. Parvovirus B19 as a cause of acquired chronic pure red cell aplasia. Br J Haematol 1994; 87:818
Presentacin Clnica
Insidioso
Insidioso
Ausencia
Ausencia de
de
precursores
precursores
eritroides
eritroides
No
No hallazgos
hallazgos
fsicos
fsicos
caracteristicos*
caracteristicos*
MO
MO celularidad
celularidad
normal
normal
Morfologa
Morfologa de
de
eritrocitos normal
eritrocitos
normal
Transferrina
Transferrina
aa
casi
casi 100%
100%
TIBC
TIBC
00
PFH
PFH normales
normales
Concentraciones
Concentraciones
de
hierro
de hierro
Algoritmo Diagnostico
Tratamiento
1ra opcin
2da opcin
3era
Prednisona
60mg/dia
Ciclosporina
200-600mg
10mg/Kg
Ciclofosfamida
50mgs/da
2-3mg/kg/da
Stanley L Schrier , et al. Pure red cell aplasia in the adult Up to date Jul, 28 2015
Tasa de
respuesta
Global:30-62%
TRG: 65-85%
TRG:56%
Tratamiento Combinado
Ken-ichi Sawada, et al. Long-term outcome of patients with acquired primary idiopathic pure red cell aplasia receiving cyclosporine A. A nationwidecohort study in Japan for the PRCA
Collaborative Study Group Haematologica 2007
GLOBULINA ANTI-TIMOCITO
Janis L.Abkowitz. Pure Red Cell Aplasia: Response to Therapy With Ant i-Th ymocyte Globulin. Am J Hematolofy1986
Rituximab
S.A. Mahajan ,Rituximab: A viable treatment option for epoetin-induced pure red cell aplasia ,2015.
Idioptico:
72 pts
Timoma:
41pts
LGL: 41pts
Hirokawa M1, Sawada K, Fujishima N, Teramura M, Bessho M, Dan K. Long-term outcome of patients with acquired chronic pure red cell aplasia (PRCA) following
immunosuppressive therapy: a final report of the nationwide cohort study in 2004/2006 by the Japan PRCA collaborative study group. Br J Haematol. 2015 Mar 25. doi:
10.1111/bjh.13376. [Epub ahead of print]
Hirokawa M1, Sawada K, Fujishima N, Teramura M, Bessho M, Dan K. Long-term outcome of patients with acquired chronic pure red cell aplasia (PRCA) following
immunosuppressive therapy: a final report of the nationwide cohort study in 2004/2006 by the Japan PRCA collaborative study group. Br J Haematol. 2015 Mar 25. doi:
10.1111/bjh.13376. [Epub ahead of print]
Timectomia
Tasa de respuesta del 25 a 30%. (Zeok et al,
1979)
Puede representar un riesgo para el desarrollo de
enfermedades autoinmunes sistmicas. (Gerli
et,al 1999).
Insuficiente para la normalizacin de la
eritropoyesis en 13 pacientes.(Thompson and
Steensma,2006)
Koyama RV1, Silva LF2, Henriques VB3, Tran C4, Yoshikawa GT3. Pure red cell aplasia associated with Systemic Lupus
Erythematosus. ACTA REUMATOL PORT. 2014;39:265-268
Pronostico
Hasta el 14% remisin espontanea (embarazo y drogas)
Curso crnico
Remisiones de curso variable de 3 meses hasta 8 aos
Idioptica SG hasta 10 aos