Granulocytic sarcoma in a non-leukemic patient presenting with unusual cutaneous paraneoplastic syndrome.

40-year old man was admitted to our Institute because of an erythematous, exfoliative dermatitis, along with a gross right leg and scrotal edema. Histologic examination of the skin biopsy was consistent with a diagnosis of acquired ichthyosis (Figure 1). A total body computed tomography documented the presence of a voluminous mass in the right iliac fossa (Figure 2). Histologic, histochemical and immunohistochemical examinations of a CT-guided biopsy (Figures 3-4) were consistent with the diagnosis of granulocytic sarcoma.1 Support therapy was started, but after 8 days the patient die

Succeeding onset of hepatic, splenic, and renal infarction in polyarteritis nodosa.

TO THE EDITOR: Polyarteritis nodosa (PAN) is a systemic vasculitis involving medium-sized vessel, characterized by focal, panmural, necrotizing, and inflammatory lesions with a predilection for the vessel bifurcation (1). Although PAN may affect any organ with vascular and thromboembolic manifestations with consequent tissue ischemia, liver and splenic infarction are rarely described (2, 3). We report the extraordinary case of a 65-yr-old man presenting with severe abdominal pain, in which the sudden onset of contemporary, massive liver, and splenic infarction was the first manifestation of an underlying PAN. Interestingly, after a few days of hospitalization, the patient also developed bilateral renal infarctions

Noninvasive assessment of myocardial involvement in patients with systemic sclerosis: role of signal averaged electrocardiography.

Objective. To assess the role of late ventricular potentials (LVP) in detecting early myocardial involvement in patients with systemic sclerosis (SSc). Methods. Seventy-seven patients with SSc (68 women, 9 men, mean age 50 ± 13 yrs) and 33 control subjects (18 women, 15 men, mean age 46 ± 10 yrs) underwent resting electrocardiogram (ECG), 24 h Holter monitoring, complete echocardiographic and Doppler echocardiographic examination, and signal averaged ECG at high pass setting of 40 Hz, with the low pass fixed at 250 Hz. Patients with SSc underwent resting myocardial scintigraphy and radionuclide angioventriculography. Results. The prevalence of LVP at 40 Hz was 20.5%. Compared to control subjects, patients with SSc showed higher prevalence of septal infarction pattern (p = 0.05), complex ventricular arrhythmias (p = 0.03), pulmonary arterial hypertension (p < 0.001), and LVP (p = 0.02). Forty-four patients with SSc (57.1%) had resting perfusion defects by myocardial scintigraphy Fourteen of 15 patients with LVP showed perfusion defects compared to 29 of 58 without LVP (p = 0.002). Linear regression analysis showed that myocardial perfusion defect score was significantly correlated to either the filtered QRS duration, or the duration of low amplitude signals < 40 μV of the terminal QRS, or the root mean square voltage of the last 40 ms of the QRS complex. After a mean followup period of 20 months, 8 patients died. In 2 patients who died suddenly, LVP were present. Conclusion. Signal averaged ECG is a sensitive and inexpensive technique in the clinical assessment and followup of patients with SSc

Cardiac involvement in patients with Wegener's granulomatosis

Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis, which could potentially affect any organ system. However, there have only been a few reports on cardiac involvement. We described the echocardiographic findings in nine patients affected by WG. A complete M-mode, two-dimensional, Doppler and color-Doppler transthoracic echocardiogram was performed in nine patients (seven females and two males) affected by WG. In each patient, cardiac abnormality, for example, valvular damage, left ventricular global systolic dysfunction, or pericardial effusion, was detected. In particular, heart valve disease was found in eight patients, and in three cases, aortic valve insufficiency, which was severe enough to require surgical valve replacement, was observed. Cardiac involvement in patients with WG is common. In particular, there is a high frequency of aortic valve abnormalities. Thus, an echocardiographic study should be routinely performed