Aggressive Systemic Mastocytosis in Association with Pure Red Cell Aplasia

Aggressive systemic mastocytosis (ASM) is characterized by mast cell accumulation in systemic organs. Though ASM may be associated with other hematological disorders, the association with pure red cell aplasia (PRCA) is rare and has not been reported. Pure red cell aplasia (PRCA) is a syndrome, characterized by normochromic normocytic anemia, reticulocytopenia, and severe erythroid hypoplasia. The myeloid and megakaryocytic cell lines usually remain normal. Here, we report an unusual case of ASM, presenting in association with PRCA and the management challenges.

• Publication year

  2018

• Venue

  Case Reports in Hematology

• Publication date

  2018-07-08

• Fields of study

  Medicine

• Identifiers
  DOI 10.1155/2018/6928571PMID 30069418PMCID 6057401
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• High-dose steroids as a therapeutic option in the management of spur cell haemolytic anaemia

  2018cited by this paper
• The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.

  2016cited by this paper
• Efficacy and Safety of Midostaurin in Advanced Systemic Mastocytosis.

  2016cited by this paper
• Additional mutations in SRSF2, ASXL1 and/or RUNX1 identify a high-risk group of patients with KIT D816V+ advanced systemic mastocytosis

  2016cited by this paper
• Treatment of CD30-positive systemic mastocytosis with brentuximab vedotin.

  2016cited by this paper
• Marked hyperferritinemia does not predict for HLH in the adult population.

  2015cited by this paper
• Hematopoietic stem-cell transplantation for advanced systemic mastocytosis.

  2014cited by this paper
• Presentation, diagnosis, and management of mast cell activation syndrome

  2013cited by this paper
• Anti CD-30 Antibody-Drug Conjugate Brentuximab Vedotin (ADCETRIS®) May Be a Promising Treatment Option for Systemic Mastocytosis (SM).

  2012cited by this paper
• Single Nucleotide Polymorphism Array Lesions, TET2, DNMT3A, ASXL1 and CBL Mutations Are Present in Systemic Mastocytosis

  2012cited by this paper
• Mast cell activation disorder masquerading as pure red cell aplasia

  2010cited by this paper
• Cytoreductive therapy in 108 adults with systemic mastocytosis: Outcome analysis and response prediction during treatment with interferon‐alpha, hydroxyurea, imatinib mesylate or 2‐chlorodeoxyadenosine

  2009cited by this paper
• Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis.

  2009cited by this paper
• Diagnosis and management of acquired pure red cell aplasia.

  2009cited by this paper
• Acquired pure red cell aplasia: updated review of treatment

  2008cited by this paper
• Treatment of systemic mast cell disease: beyond interferon.

  2004cited by this paper
• Response to therapy with interferon alpha-2b and prednisolone in aggressive systemic mastocytosis: report of five cases and review of the literature.

  2004cited by this paper
• Aggressive systemic mastocytosis and related mast cell disorders: current treatment options and proposed response criteria.

  2003cited by this paper
• Diagnostic criteria and classification of mastocytosis: a consensus proposal.

  2001cited by this paper
• Treatment of systemic mast cell disorders.

  2000cited by this paper
• Serum tryptase and the laboratory diagnosis of systemic mastocytosis.

  2000cited by this paper
• Classification and diagnosis of mastocytosis: current status.

  1991cited by this paper
• Classification and diagnosis of mastocytosis: current status.

  1991cited by this paper
• The treatment of mastocytosis: an overview.

  1991cited by this paper
• The treatment of mastocytosis: an overview.

  1991cited by this paper
• Intervention with epinephrine in hypotension associated with mastocytosis.

  1983cited by this paper

• Effects of Primary Mast Cell Disease on Hemostasis and Erythropoiesis

  2021cites this paper