Hematology and Oncology Book

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Sideroblastic AnemiaAka: Pearson Syndrome

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  1. See Also
    1. Microcytic Anemia
  2. Pathophysiology
    1. Defective heme metabolism
  3. Causes
    1. Toxin Exposure
      1. Alcohol Abuse
      2. Lead exposure
      3. Isoniazid
      4. Chloramphenicol
    2. Neoplasm or inflammatory disease
      1. Carcinoma
      2. Leukemia
      3. Lymphoma
      4. Rheumatoid Arthritis
    3. X-Linked recessive Sideroblastic Anemia
    4. Pearson Syndrome
      1. Vacuolation of marrow precursor cells
      2. Exocrine pancreatic dysfunction
  4. Labs
    1. Complete Blood Count
      1. See Hemoglobin Cutoffs for Anemia
      2. See Hematocrit Cutoffs for Anemia
      3. Mean Corpuscular Volume (MCV) <80 fl
        1. See MCV Cutoffs for Microcytic Anemia
        2. MCV cutoff varies by age and per reference
    2. Serum Iron increased
    3. Peripheral Smear
      1. Hypochromic Microcytic Anemia
    4. Bone Marrow Aspirate
      1. Ringed Sideroblasts
  5. Management
    1. Remove offending exposure
    2. Pyridoxine (variable efficacy)

Pearson's syndrome (C0342773)

ConceptsDisease or Syndrome (T047)
EnglishPEARSON MARROW-PANCREAS SYNDROME, Pearson's syndrome, SIDEROBLASTIC ANEMIA WITH MARROW CELL VACUOLIZATION AND EXOCRINE PANCREATIC DYSFUNCTION
Spanishsindrome de Pearson
Parent ConceptsAnemia, Sideroblastic (C0002896), Congenital anemia (C0158995), Disorder of pyruvate metabolism and mitochondrial respiratory chain (C0342766)
SourcesOMIM, PDQ, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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