Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 12, Number 12, December 2021, pages 474-480

Successful Treatment of Thrombocytopenia, Anasarca, Fever, Reticulin Myelofibrosis/Renal Insufficiency, and Organomegaly Syndrome Using Plasma Exchange Followed by Rituximab in the Intensive Care Unit


Figure 1.
Figure 1. Time course of treatment in the general ward. Antibiotics were administered during the first week after admission; however her inflammatory markers remained elevated and her fever persisted. Anasarca with fluid retention showed extreme progression, and a blood examination revealed thrombocytopenia and developed renal dysfunction. She was diagnosed as TAFRO syndrome. Diuretics had no effect on her fluid retention, steroid pulse therapy followed by steroid maintenance therapy and hemodialysis was performed in an attempt to improve her fluid retention. However, no therapeutic effect was obtained. Finally, she was admitted to the ICU. TAFRO: thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal insufficiency, and organomegaly; HD: hemodialysis; PSL: prednisolone; CTRX: ceftriaxone; MEPM: meropenem; BT: body temperature; Cr: creatinine; Plt: platelet; ICU: intensive care unit.
Figure 2.
Figure 2. Time course of treatment in the ICU. Mechanical ventilation support was required after endotracheal intubation due to respiratory failure, and CRRT was performed due to renal failure. PE was performed using albumin replacement for 2 days and fibrinogen replacement for 4 days. The rapid initiation of PE was very effective. Her fever quickly declined, her inflammatory reaction decreased, and her anasarca with fluid retention showed a dramatic improvement. After the beneficial effects of PE, rituximab was initiated. PE: plasma exchange; CRRT; continuous renal replacement therapy; RBC: red blood cell; Plt: platelet; FFP: fresh frozen plasma; PSL: prednisolone; NAD: noradrenaline; VCM: vancomycin; PIPC/TAZ: tazobactam/piperacillin; MEPM: meropenem; CRP: C-reactive protein; ICU: intensive care unit.
Figure 3.
Figure 3. Abdominal computed tomography scan during hospitalization. (a) On admission. (b) Fourteen days after admission. (c) Twenty-four days after admission. (d) Thirty-three days after admission. Anasarca with fluid retention showed extreme progression before ICU admission (until 24th day after admission). After the treatment of plasma exchange, her anasarca with fluid retention showed a dramatic improvement. ICU: intensive care unit; PE: plasma exchange.


Table 1. Time Course of Her Laboratory Data
General wardICU
On admission (day 1)On diagnosis (day 17)On ICU admission (day 24)Before PE (day 25)After PE (day 33)Before rituximab (day 34)After rituximab (day 42)
Bone marrow biopsy; M-protein (-). ICU: intensive care unit; PE: plasma exchange; WBC: white blood cell; RBC: red blood cell; AST: aspartate aminotransferase; ALT: alanine aminotransferase; LDH: lactate dehydrogenase; ALP: alkaline phosphatase; γ-GTP: γ-glutamyltranspeptidase; BUN: blood urea nitrogen; CRP: C-reactive protein; ANA: antinuclear antibody; IgG: Immunoglobulin G; IgM: Immunoglobulin M; IgA: Immunoglobulin A; sIL-2R: soluble interleukin-2 receptor; IL-6: Interleukin-6; VEGF: vascular endothelial growth factor; CMV: cytomegalovirus; EBV VCA: Epstein-Barr virus virus capsid antigen; HIV: human immunodeficiency virus.
WBC (× 103/µL)6.214.614.620.22515.55.9
RBC (× 106/µL)4.493.792.993.713.333.13.1
Hemoglobin (g/dL)13.510.711.210.410.19.49.9
Hematocrit (%)39.832.733.631.83329.831.3
Platelet (× 103/µL)87153943526569
Total protein (g/dL)
Albumin (g/dL)
AST (U/L)40128126117342825
ALT (U/L)28336158222531
LDH (U/L)255271250276179199199
ALP (U/L)258382342340342241
γ-GTP (U/L)393138363560
Total bilirubin (mg/dL)5.14.432.
BUN (mg/dL)30651261077410778
Creatinine (mg/dL)1.551.692.652.491.52.11.54
CRP (mg/dL)16.7518.1617.1715.350.260.310.18
Ferritin (ng/mL)429.5
ANA (speckled)160
IgG (mg/dL)1,560
IgA (mg/dL)384
IgM (mg/dL)71
sIL-2R (U/mL)761
IL-6 (pg/mL)38
VEGF (pg/mL)219
CMV IgM(-)