Lab Med Online.  2019 Oct;9(4):249-253. 10.3343/lmo.2019.9.4.249.

Therapeutic Plasma Exchange in a Patient with Hemophagocytic Lymphohistiocytosis

  • 1Department of Laboratory Medicine, Yonsei University College of Medicine, Seoul, Korea.
  • 2Department of Laboratory Medicine, Serim Hospital, Incheon, Korea.
  • 3Department of Laboratory Medicine, Severance Hospital, Seoul, Korea.


A 22-year old female patient with systemic lupus erythematosus presenting microangiopathic hemolytic anemia was treated with therapeutic plasma exchange 23 times. The patient's condition and laboratory findings (aspartate aminotransferase, alanine aminotransferase, ferritin, total bilirubin, and lactate dehydrogenase) did not improve despite the initial 18 therapeutic plasma exchange treatments. Thrombotic thrombocytopenic purpura was ruled out due to normal ADAMTS-13 activity test result; hemophagocytic lymphohistiocytosis was diagnosed based on fever, splenomegaly, pancytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow aspiration. The patient's condition improved rapidly upon treatment with a combination of immunosuppressants and cytotoxic agents, and more therapeutic plasma exchanges were performed five consecutive times with prolonged intervals in between. We observed that therapeutic plasma exchange treatment alone was not effective enough to treat hemophagocytic lymphohistiocytosis, unlike thrombotic thrombocytopenic purpura. Therefore, it is necessary to determine and start drug administration promptly in the treatment of hemophagocytic lymphohistiocytosis with thrombotic microangiopathy.


Hemophagocytic lymphohistiocytosis; Therapeutic plasma exchange; Thrombotic microangiopathy

MeSH Terms

Alanine Transaminase
Anemia, Hemolytic
Bone Marrow
Immunosuppressive Agents
Lactic Acid
Lupus Erythematosus, Systemic
Lymphohistiocytosis, Hemophagocytic*
Plasma Exchange*
Purpura, Thrombotic Thrombocytopenic
Thrombotic Microangiopathies
Alanine Transaminase
Immunosuppressive Agents
Lactic Acid


  • Fig. 1 Peripheral blood smear shows moderate anisocytosis with mild fragment RBCs (arrows) at the time of admission (Wright Giemsa stain, ×1,000; scale bar, 100 µm).

  • Fig. 2 Hemophagocytosis in bone marrow aspiration smear (Wright Giemsa stain, ×1,000; scale bar, 100 µm).

  • Fig. 3 Changes of laboratory data following therapeutic plasma exchange and drug therapy. (A) Platelet count and hemoglobin levels remained at around the same level. (B) Ferritin and LDH levels increased in the early stage and decreased after administration of the immunosuppressant and cytotoxic medication. (C) Triglyceride and total bilirubin levels increased, despite TPE. Thick arrows below the x-axis indicate the days of therapeutic plasma exchange (TPE) procedures. Vertical dotted line in the graphs indicates the time of medication (immunosuppressant and cytotoxic agent).


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