Primary pyomyositis is a pyogenic infection of skeletal muscle with abscess formation, which traditionally lacks an identifiable cause. We present a case of ...
Ann R Coll Surg Engl 2002; 84: 26-28
The Royal College of Surgeons of England
Case report
Cyclic neutropenia and pyomyositis: a rare cause of overwhelming sepsis MD Waites, JV Roberts, D Scott-Coombes, S Al-Hamali Department of Surgery, King's College Hospital, London, UK Primary pyomyositis is a pyogenic infection of skeletal muscle with abscess formation, which traditionally lacks an identifiable cause. We present a case of pyomyositis for which a cause was established. This was largely due to the fact that the patient was young and fit, enabling him to survive such overwhelming sepsis long enough for cycling of his neutrophil count to become apparent. Having had multiple abscesses drained, he was successfully treated with granulocyte colony stimulating factor and has remained well since. Key words: Pyomyositis Cyclic neutropenia Granulocyte colony stimulating factor -
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previously fit 31-year-old married Caucasian man presented as an emergency with a 10-day history of worsening pain in the right thigh and buttock, myalgia, increasing jaundice and one episode of fever with rigors. He had been anuric for 18 h and gave no history of recent foreign travel, intravenous drug abuse or sexual promiscuity. On examination, he was jaundiced, apyrexial but tachycardic and hypotensive. Examination of the musculoskeletal system revealed swollen erythematous elbows with a decreased range of movement on the right, and swelling of the left hand and forearm. Movement of the right hip was severely restricted and there was limited flexion of the right knee. There was also bilateral calf swelling. The rest of the exanidnation was normal. Investigations on admission are summarised in Table 1. His septicaemia and hepatorenal failure were treated with broad-spectrum antibiotics (ciprofloxacin, flucloxacillin, amoxycillin and metronidazole) and haemofiltration. A computerized tomography (CT) scan revealed a right psoas abscess. This was incised and drained of 20 ml of pus, which A
Table 1 Investigations on admission 12.0 g.dl-1 9.4 x 109.1-l 91 x 109.1-1
Hb (Haemoglobin) WCC (White cell count) Plts (Platelets) Na+ (Sodium) K+ (Potassium) Urea (Urea) Creat (Creatine)
129 mmol.l-1 5.1 mmol.l-1 44 mmol.l-1 336 gmol.l-1
Bil (Bilirubin) Prot (Total Protein) Alb (Albumen) CRP (C-reactive protein)
246 jmol.l-1 65 g.1-' 27 g.l203 mg.l-1
AST (Aspartate-amino transferase) ALP (Alkaline phosphatase) ALT (Alanine-amino transferase) GGT (Gamma-glutamyl transferase)
Blood cultures HIV(Human Immunodeficiency Virus) serology Hepatitis serology
38 IU.l-1 254 82 74
Negative Negative Negative
Correspondence to: Mr MD Waites, Department of Surgery, King's College Hospital, Denmark Hill, London SE5 9RS, UK 26
Ann R Coll Surg Engl 2002; 84
CYCLIC NEUTROPENIA AND PYOMYOSITIS: A RARE CAUSE OF OVERWHELMING SEPSIS
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Figure 1 Plot of neutrophil count against time with arrows indicating organisms cultured and antibiotic therapy.
cultured Staphylococcus aureus. Despite selective antimicrobial therapy (flucloxacillin, fusidin and metronidazole), he remained ill, requiring haemofiltration, ventilation and inotropic support. A further CT scan revealed intramural gas of the terminal ileum and some free intra-abdominal fluid. Exploratory laparotomy found a small amount of straw-coloured fluid, but no abscess or diseased bowel. Three weeks later, he became pyrexial and subsequently developed multiple abscesses on all four limbs, which required incision and drainage. In addition, he developed a hypopian of his right eye which ulcerated. This was followed by a less severe ulceration of the left cornea. Pseudomonas aeruginosa was cultured from the right lower limb abscess and comeal scrapings. Despite drainage of all apparent abscesses, the patient remained febrile and unwell. A white cell scan identified an abscess around the left knee joint, from which 100 ml of sterile pus was drained. On review of blood counts, it became apparent that there was cycling of the absolute neutrophil count (Fig. 1). Bone marrow aspirate demonstrated a prominent left-shifted granulopoeisis with minor dysplastic features in the erythroid series consistent with chronic disease. Anti-neutrophil antibodies and neutrophil function induding chemotaxis and phagocytosis all proved normal. A diagnosis of cyclic neutropenia was established from daily full blood counts, with a Ann R Coll Surg Engl 2002; 84
white cell differential. This was confirmed by noting an improvement in neutrophil count and symptoms once treatment with granulocyte colony stimulating factor (GCSF) was commenced. Two and a half months after admission, he made sufficient recovery to be discharged home. Discussion
This case report illustrates how a fit young man can rapidly become moribund with pyomyositis and septicaemia. The diagnosis of cyclic neutropenia provides a pathological basis for pyomyositis, which traditionally lacks identifiable causal factors.' This diagnosis was only made possible because our patient, fortunately, was young and fit enough to survive such overwhelming illness to enable fluctuations in white cell count to become apparent. Primary pyomyositis, first described by Scriba in 1885?'3 is defined as a pyogenic infection of skeletal muscle leading to abscess formation. It is more common in tropical countries where suggested predisposing factors include parasitic, leptospiral, spirochaetal and viral infections as well as nutritional deficiencies.13 27
WAITES
CYCLIC NEUTROPENIA AND PYOMYOSITIS: A RARE CAUSE OF OVERWHELMING SEPSIS
Factors that predispose to pyomyositis in non-tropical countries include HIV, diabetes, haemopoeitic disorders and old age, owing to impaired neutrophil function.3 In this case, it would appear that severe neutropenia predisposed to the patient developing pyomyositis. Human cyclic neutropenia is a rare haematological disorder with an estimated incidence of 0.5-1 case per million population." It usually presents in childhood, although in up to 25% of well-documented cases the disease becomes dinically evident after the age of 20 years. In such cases, the disease tends to be associated with a distinct febrile illness with prostration.7 Cyclic neutropenia is characterised by regular oscillations in blood neutrophil counts which are reported to vary between 15 and 35 days, although the majority of cases reported have cycles every 19-23 days.4 Cyclic neutropenia is hereditary, although familial cases are too isolated to imply any definitive mode of inheritance.4 Indeed, there was no discernible family history in this case. An abnormality of the negative feedback regulation of granulopoeisis itself partly accounts for the neutropenia.4 However, the pathogenesis of the cydic nature of this illness remains obscure.
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Current treatrnent of cyclic neutropenia favours GCSF on alternate days as a means of over-riding the dysfunctional negative feedback mechanism of granulopoeisis. Our patient has remained well since being commenced on GCSF and will remain under haematological follow-up, possibly requiring GCSF for the rest of his life. References 1. Chiedozi LC. Pyomyositis. Review of 205 cases in 112 patients. Am J Surg 1979; 137: 255-9. 2. Gibson RK, Rosenthal SS, Lukert BP. Pyomyositis. Increasing recognition in temperate climates. Am J Med 1984; 77: 768-72. 3. Gomez-Revio JJ, Azner nI. Pablos JL, Diaz-Gonzalez F, Lafron A. Non tropical pyomyositis in adults. Semin Arthritis Rheum 1994; 23: 396 405. 4. Wright D, Dale D. Human cyclic neutropenia - clinical review and longterm follow up of patients. Medicine 1981; 60:1-13. 5. Dale D, Hammond W. Cyclic neutropenia: a clinical review. Blood Rev 1988; 2: 178-85. 6. Welte K, Dale D. Pathophysiology and treatment of severe chronic neutropenia. Ann Haematol 1996; 72:158-65. 7. Bandason C, Lee RJ. Fatal human cyclic neutropenia with unresolving tonsillitis and bilateral cervical abscesses. J Laryngol Otol 1991; 105: 487-9. 8. Sievers EL, Dale D. Non-malignant neutropenia. Blood Rev 1996; 10: 95-100.
Ann R Coll Surg Engl 2002; 84
