Pancytopenia

張貼日期：2018/3/7 上午 11:30:56

Etiology

• 第一種分析法

  • Primary BM disease

    • Hypercellular

      • Lymphoma with BM involvement

      • Leukemia (aleukemic)

      • Multiple myeloma

      • Myelodysplasia (MDS)

      • Paroxysmal nocturnal hemoglobinuria (PNH)

    • Hypocellular

      • Aplastic anemia

      • Hypoplastic MDS

    • Fibrosis: primary myelofibrosis, secondary to malignancy or systemic disease

  • Secondary to systemic disease

    • Vitamin B12 / folic deficiency

    • Alcoholism

    • Drugs (如下附表1)

    • Hypersplenism

    • Infection

      • Severe sepsis

      • HIV, CMV, EBV

      • TB

    • Autoimmune

      • SLE

      • Adult-onset Still’s disease (AOSD)

    • Hemophagocytosis

• 附表1. Drugs associated with pancytopenia

• 第二種分析法

  • Bone marrow infiltration/replacement

    • Malignant

      • Acute leukemias

      • Chronic leukemias/myeloproliferative neoplasms (MPN)

      • Myelodysplastic syndromes (MDS)

      • Multiple myeloma

      • Metastatic cancer

    • Non-malignant

      • Myelofibrosis

      • Infectious (eg, fungal, tuberculous)

      • Storage diseases

  • Bone marrow failure

    • Immune destruction/suppression

      • Aplastic anemia/paroxysmal nocturnal hemoglobinuria

      • Medications¶

        • Cytotoxic drugs

        • Idiosyncratic reactions to medications

      • Large granular lymphocyte leukemia

        • Autoimmune disorders (eg, systemic lupus erythematosus [SLE], rheumatoid arthritis [RA], sarcoidosis)

      • Hemophagocytic lymphohistiocytosis (HLH)

    • Nutritional

      • Megaloblastic (vitamin B12, folate)

      • Excessive alcohol

      • Other (eg, copper deficiency, zinc toxicity)

      • Malnutrition/anorexia nervosa with gelatinous degeneration

    • Marrow suppression

      • Viral infection (eg, HIV, hepatitis, Epstein-Barr virus [EBV])

    • Ineffective hematopoiesis (eg, MDS, nutritional)

  • Destruction/sequestration/redistribution

    • Consumption

      • Disseminated intravascular coagulation (eg, associated with sepsis, acute promyelocytic leukemia) 

    • Splenomegaly

      • Portal hypertension/cirrhosis

      • Infections (eg, EBV)

      • Autoimmune disorders (eg, SLE, RA/Felty syndrome)

      • Malignancies (eg, lymphomas, MPN)

      • Myelofibrosis with myeloid metaplasia

      • Storage diseases (eg, Gaucher)

1. Blood test: Reticulocyte, Bil-T/-D, LDH, Ferritin, Iron, TIBC, TG, ANA, anti-ENA, C3, C4, RF, Vitamin B12, Folic acid, serum IFE, HIV-combo test, EBV viral load, HBsAg, anti-HCV, PNH

2. BM study: A+B 

備註:

1. 若anti-ENA(+) 再加抽ENA細項Anti-dsDNA, Anti-SSA/SSB antibody, Scl-70 Ab, Sm/RNP, 抗Ribosomal-P抗體, Anti-Jo-1

2. Vitamin B12 血中濃度對於Vitamin B12缺乏的sensitivity不是太好，建議搭配methylmalonic acid, homocysteine濃度來診斷