Cancer-associated microangiopathic hemolytic anemia with thrombocytopenia

An important diagnostic consideration

Michelle A. Elliott, Louis Letendre, Dennis A. Gastineau, Jeffrey L. Winters, Rajiv K. Pruthi, John A. Heit

Research output: Contribution to journalArticle

40 Citations (Scopus)

Abstract

Background: Early initiation of plasma exchange (PE) allows more than 80% of patients with idiopathic thrombotic thrombocytopenic purpura (TTP), most commonly because of severe ADAMTS13 deficiency, to achieve remission and mandates urgency in diagnosis and therapy. Metastatic cancer may present with a microangiopathic hemolytic anemia with thrombocytopenia that is clinically similar to TTP but does not respond to PE. ADAMTS13 activity can be diagnostic but usually not immediately available. Recognition of cancer-associated microangiopathic hemolytic anemia with thrombocytopenia (CA-MHA) is paramount to avoid inappropriate PE therapy and delays in cancer-specific chemotherapy. Objective: To identify distinguishing characteristics of CA-MHA and TTP to facilitate early recognition of CA-MHA. Methods: In a retrospective cohort study, baseline clinical and laboratory data of consecutive adult patients with CA-MHA (n = 7) or autoimmune TTP (n = 7) from a registry of patients with clinically suspected acute TTP referred for PE were compared. Results: The frequencies of bone pain and respiratory symptoms were significantly greater among patients with CA-MHA compared to patients with TTP; other baseline clinical and laboratory characteristics did not differ significantly between the two groups. Response to PE and mortality at day 30 were significantly worse for CA-MHA (14% and 71%, respectively) compared to patients with TTP (86% and 14%, respectively). Conclusions: Baseline clinical and laboratory characteristics largely do not distinguish acute CA-MHA from autoimmune acute TTP. While all suspected acute patients TTP should receive urgent PE, bone pain, respiratory symptoms, or inadequate PE response should prompt an early search for CA-MHA.

Original languageEnglish (US)
Pages (from-to)43-50
Number of pages8
JournalEuropean Journal of Haematology
Volume85
Issue number1
DOIs
StatePublished - Jul 2010

Fingerprint

Hemolytic Anemia
Thrombotic Thrombocytopenic Purpura
Thrombocytopenia
Plasma Exchange
Neoplasms
Idiopathic Thrombocytopenic Purpura
Bone and Bones
Pain
Registries
Cohort Studies
Retrospective Studies
Drug Therapy
Mortality

Keywords

  • ADAMTS13 deficiency
  • Cancer-associated microangiopathic hemolytic anemia with thrombocytopenia
  • Thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Hematology

Cite this

Cancer-associated microangiopathic hemolytic anemia with thrombocytopenia : An important diagnostic consideration. / Elliott, Michelle A.; Letendre, Louis; Gastineau, Dennis A.; Winters, Jeffrey L.; Pruthi, Rajiv K.; Heit, John A.

In: European Journal of Haematology, Vol. 85, No. 1, 07.2010, p. 43-50.

Research output: Contribution to journalArticle

Elliott, Michelle A. ; Letendre, Louis ; Gastineau, Dennis A. ; Winters, Jeffrey L. ; Pruthi, Rajiv K. ; Heit, John A. / Cancer-associated microangiopathic hemolytic anemia with thrombocytopenia : An important diagnostic consideration. In: European Journal of Haematology. 2010 ; Vol. 85, No. 1. pp. 43-50.
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abstract = "Background: Early initiation of plasma exchange (PE) allows more than 80{\%} of patients with idiopathic thrombotic thrombocytopenic purpura (TTP), most commonly because of severe ADAMTS13 deficiency, to achieve remission and mandates urgency in diagnosis and therapy. Metastatic cancer may present with a microangiopathic hemolytic anemia with thrombocytopenia that is clinically similar to TTP but does not respond to PE. ADAMTS13 activity can be diagnostic but usually not immediately available. Recognition of cancer-associated microangiopathic hemolytic anemia with thrombocytopenia (CA-MHA) is paramount to avoid inappropriate PE therapy and delays in cancer-specific chemotherapy. Objective: To identify distinguishing characteristics of CA-MHA and TTP to facilitate early recognition of CA-MHA. Methods: In a retrospective cohort study, baseline clinical and laboratory data of consecutive adult patients with CA-MHA (n = 7) or autoimmune TTP (n = 7) from a registry of patients with clinically suspected acute TTP referred for PE were compared. Results: The frequencies of bone pain and respiratory symptoms were significantly greater among patients with CA-MHA compared to patients with TTP; other baseline clinical and laboratory characteristics did not differ significantly between the two groups. Response to PE and mortality at day 30 were significantly worse for CA-MHA (14{\%} and 71{\%}, respectively) compared to patients with TTP (86{\%} and 14{\%}, respectively). Conclusions: Baseline clinical and laboratory characteristics largely do not distinguish acute CA-MHA from autoimmune acute TTP. While all suspected acute patients TTP should receive urgent PE, bone pain, respiratory symptoms, or inadequate PE response should prompt an early search for CA-MHA.",
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AU - Heit, John A.

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