Ann Hematol (2006) 85: 629–630 DOI 10.1007/s00277-006-0126-0 LETTER TO THE EDITOR K. Ramasamy . Z. Y. Lim . M. Savvas . J. R. Salisbury . I. Dokal . G. J. Mufti . A. Pagliuca Disseminated herpes virus (HSV-2) infection with rhabdomyolysis and hemophagocytic lymphohistiocytosis in a patient with bone marrow failure syndrome Received: 24 March 2006 / Accepted: 6 April 2006 / Published online: 17 May 2006 # Springer-Verlag 2006 Dear Editor, The inherited bone marrow failure syndromes, such as Fanconi anemia, dyskeratosis congenita, and Shwachman– Diamond syndrome, are associated with higher risk of infections and development of secondary neoplasia [1]. Hemophagocytic lymphohistiocytosis (HLH) is a life- threatening syndrome initiated by a hyperstimulated immune system leading to pyrexia, splenomegaly, and profound cytopenias [2]. Although acquired HLH is most commonly associated with infections, HLH is a rarely reported complication in patients with bone marrow failure syndromes. A 33-year-old nulliparous lady had been diagnosed with a bone marrow failure syndrome at the age of 8. There was no associated family history, and clinical diagnosis was based on morphological features of macrocytosis and pancytopenia, together with phenotypic features of short stature, micrognathia, hypopigmentation of skin, and bilateral undeveloped hypothenar eminences. Results of diepoxybutane test showed no increase in chromosomal breakage, and chromosomal analysis revealed a normal 46XX karyotype. She initially required blood and platelet support, but became transfusion-independent within a year of starting oxymetholone and prednisolone. Due to the mild hematological course, it was suspected that she might be a somatic mosaic for Fanconi anemia, but she declined a skin biopsy, which could have substantiated this diagnosis. She presented to her general practitioner with a 5-day history of dysuria and was treated with antibiotics for a presumed urinary tract infection. Her symptoms persisted and she referred to our hospital. On admission, she was pyrexial with an extensive vesicular rash over her face, arms, legs, and vulval regions. Her full blood count showed: hemoglobin 12.2 g/dl, WBC 3.6×10 9 /l, platelets 98×10 9 /l, mean corpuscular volume 101 fl, (neutrophils 3×10 9 /l, lymphocytes 0.31×10 9 /l) with C-reactive protein of 147.4 g/l (normal: <5.0 g/l). Vulval examination revealed ulceration with purulent discharge, and a provi- sional diagnosis of “disseminated herpes simplex viral (HSV) infection” was made. This was confirmed when serum and vesicular fluid samples tested positive for HSV- 2 DNA. She was commenced on systemic antibacterial and antiviral agents (ceftriaxone 2 g once daily intravenously, metronidazole 500 mg thrice times daily intravenously, aciclovir 10 mg/kg thrice daily intravenously), and made steady clinical improvement. However, on the fifth day of admission, she was found collapsed and unresponsive. Investigations revealed severe pancytopenia (hemoglobin, 1.7 g/dl; WBC, 1.17×10 9 /l; neutrophils, 0.64×10 9 /l; and platelets, 1×10 9 /l), with biochemical evidence of rhabdo- myolysis and acute renal failure (creatine kinase 19,672 IU/ l, sodium 120 mmol/l, potassium 7.7 mmol/l, urea 13.3 mmol/l, creatinine 233 mmol/l). Bone marrow aspiration revealed a hypocellular bone marrow with markedly increased macrophages and evidence of triline- age hemophagocytosis (Fig. 1). A clinical diagnosis of HSV 2 associated hemophagocytosis and rhabdomyolysis was made. Unfortunately, due to the severe thrombocyto- penia, we were unable to perform a muscle biopsy to confirm the nature of the rhabdomyolysis. She developed multiorgan failure requiring systemic antiviral therapy, K. Ramasamy . Z. Y. Lim . G. J. Mufti . A. Pagliuca (*) Department of Haematological Medicine, Kings College London and Kings College Hospital, Denmark Hill, London, SE5 9RS, UK e-mail: tony.pagliuca@kingsch.nhs.uk Tel.: +44-207-3463709 Fax: +44-207-3463514 M. Savvas Department of Obstetrics & Gynaecology, Kings College London and Kings College Hospital, London, UK J. R. Salisbury Department of Histopathology, Kings College London and Kings College Hospital, London, UK I. Dokal Department of Haematology, Imperial College London and Hammersmith Hospital, London, UK