Korean J Crit Care Med.  2015 Feb;30(1):38-41. 10.4266/kjccm.2015.30.1.38.

Hemophagocytic Lymphohistiocytosis after Lung Transplantation

• Leem AY
• Moon SW
• Kim SY
• Park MS
• Kim YS
• Kim SK
• Chang J
• Paik HC
• Cheong JW
• Chung KS

• KMID: 2227700
• DOI: http://doi.org/10.4266/kjccm.2015.30.1.38

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal complication after solid organ transplantation. Acquired forms of HLH are described in association with severe sepsis, autoimmune disorders, malignancy, immune-compromised states, infections, and solid organ transplantation. We experienced a case of hemophagocytic lymphohistiocytosis after bilateral lung transplantation. Leukopenia, thrombocytopenia, and hyperbilirubinemia were noted and became aggravated 50 days after transplantation. Diagnosis of HLH was based on clinical and laboratory findings of splenomegaly, cytopenia, elevated ferritin, elevated interleukin-2 receptor, and hemophagocytosis in bone marrow. Other features such as elevated bilirubin, lactate dehydrogenase, and D-dimer which can be present in HLH were also noted. The patient was immediately treated with etoposide and dexamethasone. Despite aggressive therapy, the patient deteriorated and died. Awareness of the diagnostic criteria of HLH after lung transplantation is important for clinicians.

Keyword

hemophagocytic; lung transplantation; lymphohistiocytosis

MeSH Terms

Bilirubin
Bone Marrow
Dexamethasone
Diagnosis
Etoposide
Ferritins
Humans
Hyperbilirubinemia
Interleukin-2
L-Lactate Dehydrogenase
Leukopenia
Lung Transplantation*
Lymphohistiocytosis, Hemophagocytic*
Organ Transplantation
Sepsis
Splenomegaly
Thrombocytopenia
Transplants
Bilirubin
Dexamethasone
Etoposide
Ferritins
Interleukin-2
L-Lactate Dehydrogenase

Figure

• 

  Fig. 1. Complete blood cell count (Fig. 1A) and total bilirubin (Fig. 1B). Pancytopenia and hyperbilirubinemia became aggravated after lung transplantation. WBC: white blood cell; PLT: platelet; Hb: hemoglobin; POD: postoperative day; T. bilirubin: total bilirubin.

• 

  Fig. 2. Microscopic finding from a bone marrow biopsy specimen (Fig. 2A and Fig. 2B) and immunohistochemical staining for CD68 (Fig. 2C). The microscopic finding from the bone marrow biopsy specimen shows prominent hemosiderin deposition and multifocal fat necrosis with foamy macrophage infiltrates (Fig. 2A [x100] and Fig. 2B [x400]). Immunohistochemical staining for CD68 shows an increased number of stromal macrophages (Fig. 2C).

Reference

References

1. Janka GE, Lehmberg K. Hemophagocytic syndromes an update. Blood Rev. 2014; 28:135–42.

2. Oto T, Snell GI, Goto K, Miyoshi S. Hemophagocytic syndrome: a rare but specific complication of lung transplantation. J Thorac Cardiovasc Surg. 2010; 140:e58–9.

3. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007; 48:124–31.

4. Diaz-Guzman E, Dong B, Hobbs SB, Kesler MV, Hayes D Jr. Hemophagocytic lymphohistiocytosis after lung transplant: report of 2 cases and a literature review. Exp Clin Transplant. 2011; 9:217–22.

5. Risdall RJ, McKenna RW, Nesbit ME, Krivit W, Balfour HH Jr, Simmons RL, et al. Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer. 1979; 44:993–1002.

6. Karras A, Thervet E, Legendre C; Groupe Coopératif de transplantation d’lle de France. Hemophagocytic syndrome in renal transplant recipients: report of 17 cases and review of literature. Transplantation. 2004; 77:238–43.