Refractory immune hemolytic anemia with a high thermal amplitude, low affinity igg anti-pra cold autoantibody

R. R. Ramos; B. R. Curtis; J. E. Sadler; C. S. Eby; H. Chaplin

doi:10.3109/08916939209150322

Refractory immune hemolytic anemia with a high thermal amplitude, low affinity igg anti-pr_a cold autoantibody

R. R. Ramos, B. R. Curtis, J. E. Sadler, C. S. Eby, H. Chaplin

Research output: Contribution to journal › Article › peer-review

10 Scopus citations

Abstract

A 54 y.o. woman presented with acute Coombs-negative hemolytic anemia at an outside hospital where she received 25 RBC transfusions and did not respond to prednisone or splenectomy. On transfer to our hospital, routine DAT and IAT were weakly positive, occasionally negative. When a modified "cold" antiglobulin test was employed, the result was strongly positive for IgG, weakly positive for C3d. Cold agglutinin titer was 32, and the Donath-Landsteiner test was negative. The autoantibody exhibited Pr_a specificity. The patient failed IV-IgG, high dose IV pulse steroids and cyclophosphamide, and continued to require daily transfusions. She responded 21 days after receiving daily plasma exchange (x3), with pulse cyclophosphamide on the third day, followed by escalating daily oral cyclophosphamide.

Original language	English
Pages (from-to)	149-154
Number of pages	6
Journal	Autoimmunity
Volume	12
Issue number	2
DOIs	https://doi.org/10.3109/08916939209150322
State	Published - 1992

Keywords

Low affinity autoantibody
Refractory autoimmune hemolytic anemia
Wide thermal amplitude autoantibody

Access to Document

10.3109/08916939209150322

Cite this

@article{97e24eddca464cf89c143cdd4eb88b7d,

title = "Refractory immune hemolytic anemia with a high thermal amplitude, low affinity igg anti-pra cold autoantibody",

abstract = "A 54 y.o. woman presented with acute Coombs-negative hemolytic anemia at an outside hospital where she received 25 RBC transfusions and did not respond to prednisone or splenectomy. On transfer to our hospital, routine DAT and IAT were weakly positive, occasionally negative. When a modified {"}cold{"} antiglobulin test was employed, the result was strongly positive for IgG, weakly positive for C3d. Cold agglutinin titer was 32, and the Donath-Landsteiner test was negative. The autoantibody exhibited Pra specificity. The patient failed IV-IgG, high dose IV pulse steroids and cyclophosphamide, and continued to require daily transfusions. She responded 21 days after receiving daily plasma exchange (x3), with pulse cyclophosphamide on the third day, followed by escalating daily oral cyclophosphamide.",

keywords = "Low affinity autoantibody, Refractory autoimmune hemolytic anemia, Wide thermal amplitude autoantibody",

author = "Ramos, {R. R.} and Curtis, {B. R.} and Sadler, {J. E.} and Eby, {C. S.} and H. Chaplin",

note = "Funding Information: This work was supported in part by PHS Grant CA02198 from the National Cancer Institute, and by Grant HL14147 (SCOR and Thrombosis) from the National Heart Lung Blood Institute. Department of HHS. Dr Sadler is an Associate Investigator. Howard Hughes Medical Institute.",

year = "1992",

doi = "10.3109/08916939209150322",

language = "English",

volume = "12",

pages = "149--154",

journal = "Autoimmunity",

issn = "0891-6934",

number = "2",

}

TY - JOUR

T1 - Refractory immune hemolytic anemia with a high thermal amplitude, low affinity igg anti-pra cold autoantibody

AU - Ramos, R. R.

AU - Curtis, B. R.

AU - Sadler, J. E.

AU - Eby, C. S.

AU - Chaplin, H.

N1 - Funding Information: This work was supported in part by PHS Grant CA02198 from the National Cancer Institute, and by Grant HL14147 (SCOR and Thrombosis) from the National Heart Lung Blood Institute. Department of HHS. Dr Sadler is an Associate Investigator. Howard Hughes Medical Institute.

PY - 1992

Y1 - 1992

N2 - A 54 y.o. woman presented with acute Coombs-negative hemolytic anemia at an outside hospital where she received 25 RBC transfusions and did not respond to prednisone or splenectomy. On transfer to our hospital, routine DAT and IAT were weakly positive, occasionally negative. When a modified "cold" antiglobulin test was employed, the result was strongly positive for IgG, weakly positive for C3d. Cold agglutinin titer was 32, and the Donath-Landsteiner test was negative. The autoantibody exhibited Pra specificity. The patient failed IV-IgG, high dose IV pulse steroids and cyclophosphamide, and continued to require daily transfusions. She responded 21 days after receiving daily plasma exchange (x3), with pulse cyclophosphamide on the third day, followed by escalating daily oral cyclophosphamide.

AB - A 54 y.o. woman presented with acute Coombs-negative hemolytic anemia at an outside hospital where she received 25 RBC transfusions and did not respond to prednisone or splenectomy. On transfer to our hospital, routine DAT and IAT were weakly positive, occasionally negative. When a modified "cold" antiglobulin test was employed, the result was strongly positive for IgG, weakly positive for C3d. Cold agglutinin titer was 32, and the Donath-Landsteiner test was negative. The autoantibody exhibited Pra specificity. The patient failed IV-IgG, high dose IV pulse steroids and cyclophosphamide, and continued to require daily transfusions. She responded 21 days after receiving daily plasma exchange (x3), with pulse cyclophosphamide on the third day, followed by escalating daily oral cyclophosphamide.

KW - Low affinity autoantibody

KW - Refractory autoimmune hemolytic anemia

KW - Wide thermal amplitude autoantibody

UR - http://www.scopus.com/inward/record.url?scp=0026645493&partnerID=8YFLogxK

U2 - 10.3109/08916939209150322

DO - 10.3109/08916939209150322

M3 - Article

C2 - 1617112

AN - SCOPUS:0026645493

VL - 12

SP - 149

EP - 154

JO - Autoimmunity

JF - Autoimmunity

SN - 0891-6934

IS - 2

ER -

Refractory immune hemolytic anemia with a high thermal amplitude, low affinity igg anti-pr_a cold autoantibody

Abstract

Keywords

Access to Document

Other files and links

Fingerprint

Cite this