Spontaneous Empyema of Joints and Staphylococcal Sepsis in a Patient with Severe Hemophilia A

Spontaneous Empyema of Joints and Staphylococcal Sepsis in a Patient with Severe Hemophilia A

W. Mondorf, L. Hovy, S. Habisreutinger and A. Hümmer

Clinical Course

A 38-year-old man with severe hemophilia A reported about a sudden onset of pain- ful swelling of his left knee during a vacation in Italy. Immediate transfusion of 4000 IU of factor VIII concentrate (Recombinate, Baxter) did not leed to any relieve. Due to further swelling and pain as well as general weakness the patient decided to return to Germany on the following day. At immediate hospital admission the pa- tient presented in a moderately reduced general condition and slightly elevated body temperature. The knee was tender and warm. Knee motion was markedly reduced due to swelling and severe pain. As factor VIII treatment did not lead to any improvement a diagnostic tab of the knee was performed showing blood and pus (Staph. areus). In spite of surgical drainage the general condition of the patient deteriorated rapidly so that intubation and intensive care treatment was necessary.

As swelling of other joints such as right knee, left elbow and right ankle was noted, further surgical drainages were performed and shock treatment on swing bed as well as intravenous antibiotic treatment with Imipenem was started. During the next days machine aided respiration improved slowly so that extubation was possible after 10 days. Factor VIII concentrate (Recombinate, Baxter) was trans- fused 3000 IU BID and reduced to 2000 IU BID after 6 days. No bleeding occurred during the time of hospitalization.

History and Further Outcome

The patient suffered from severe hemophilia A leading to hemarthrosis of knees, elbows and ankles. Most bleedings occurred in childhood and very seldom during the recent years so that on demand treatment with factor VIII concentrate was per- formed. Hepatitis C infection was treated 2001 with a 12 month period of Interferon and Ribavirin leading to a sustained viral response. HIV was negative. History prior to admission as well as clinical examination did not show any source of Staph. aure- us so that the reason leading to the hereby presented clinical course is still obscure.

In spite of successful management of knee empyema, pain and functional deterio- ration was worse in comparison to prior status so that a total knee replacement was performed one year later without any problems.

I. Scharrer/W. Schramm (Ed.)

34

Hemophilia Symposium Hamburg 2003

” Springer Medizin Verlag Heidelberg 2005

Summary

Sudden swelling of joints in patients with hemophilia A may not necessarily be due to bleeding. Especially, when factor treatment does not lead to any improvement, empyema may be present. Diagnostic puncture may be necessary as well as rapid drainage. Life threatening sepsis may occur rapidly in these situations.

206 W. Mondorf et al.

-2 -1 0 1 2 3 4 5 6 7 8 9 10 Sudden

swelling of left knee

Puncture of left knee, right knee, left elbow and right ankle

Drainage of affected joints

Recombinate 3000 IU BID Recombinate 2000 IU BID

Time of intubation

100% O

--- continuous reduction of O

concentration---

Shock therapy with swinging bed Therapy of arterial hypertension

Continuous recovery of joint swelling

Antibiotic therapy: Imipenem i.v., followed by Clindamycin p.o.

Ex- tubation

days

Fig. 1. Clinical course and therapy prior (–2 to –1) and during first 10 days of hospitalization