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History and Admission Findings: We report on the case of a young women presenting with macrohaematuria, petechiae and strong headaches., Investigations: Laboratory showed a thrombotic microangiopathy with helmet cells, increased LDH levels (>600 U/l), and thrombocytopenia (<40,000/μl)., Diagnosis, Treatment and Course: Due to strong haemolytic activity and headache with blurred vision, immediate plasma separation with fresh frozen plasma was commenced. Markedly decreased ADAMTS13 activity and detection of anti-ADAMTS13 antibodies were consistent with the diagnosis of idiopathic thrombotic thrombocytopenic purpura. In total, 11 plasma separations were required to stop disease activity. In parallel, immunosuppressive therapy using glucocorticoids was initiated. The patient was discharged from the hospital in a good general condition and with normalized laboratory findings 26 days after hospitalization., Conclusions: All patients with anemia and thrombocytopenia should be tested for haemolysis and helmet cells. An early diagnosis and initiation of necessary therapy are determining for the clinical outcome., (© Georg Thieme Verlag KG Stuttgart · New York.)

History and Admission Findings: A 65-year-old man was admitted with history of claudication symptoms and painful skin lesions of the lower legs. Physical examination showed palpable purpura of the lower legs and livedo reticularis, most marked at the forefoot and toes., Investigations: Computed tomography (CT) showed an aortic mass 2 cm above the bifurcation. This was treated after angiography with a covered stent. Biopsy of the skin lesions showed no sign of vasculitis and no cholesterol crystals., Treatment and Course: The patient was discharged and remained symptom-free for 9 months. He was readmitted at that time with recurrent complaints. CT revealed a subtotal stenosis of the aortic stent. A skin biopsy showed CD31-positive tumor cells in small arteries. Biopsy of a new osteolytic lesion in the ileum confirmed the diagnosis of angiosarcoma of the aorta. The patient decided in favor of palliative care and was discharged from the hospital., Conclusion: Primary tumors of the aorta, although they are rare, should be considered in the presence of an intravascular mass with stenosis to blood flow. A skin biopsy is easy to conduct and often leads to the final diagnosis., (Georg Thieme Verlag KG Stuttgart. New York.)

History and Clinical Findings: Three young men became ill one after the other with fever, headaches, vomiting, arthralgias and muscle pain. One day after beginning of symptoms all three patients developed a haemorrhagic rash with petechial and ecchymotic lesions most intense on distal extremities. 24 hours later patient no. 1 and 3 were in septic shock., Investigations: Laboratory tests showed signs of systemic infection, disseminated intravascular coagulation and renal failure. On the day of admission to the hospital blood cultures showed Neisseria meningitidis in patient no. 1 and 3. In patient no. 2 blood cultures were negative., Treatment and Course: Intravenous antibiotic therapy was started immediately after admission. In patient no. 1 and 3 purpura fulminans with multiple organ failure demanded intensive care treatment. Patient no. 1 recovered. Necrotic toes made amputations necessary. Patient no. 2 was never critically ill. Patient no. 3, whose course was complicated by a long lasting disseminated intravascular coagulation, died from massive cerebral bleeding 6 days after admission. Patient no. 2, who was treated with ciprofloxacin after symptoms began was never critically ill., Conclusion: Neisseria meningitidis sepsis has a high mortality rate. Rapid admission to the hospital and beginning of an antibiotic therapy with penicillin G or a third-generation cephalosporin is a priority when meningococcal disease is suspected. Chemoprophylaxis should be offered to close contacts of patients.

Over a period of 2 years recurrent erysipelas-like skin eruptions developed over both lower legs of a 77-year-old woman. On examination there was a vasculitic purpura which histologically was like leukocytoclastic vasculitis. Further tests revealed a positive rheuma factor, a mixed type II cryoglobulinaemia of monoclonal IgM-kappa paraprotein and polyclonal IgG, as well as chronic hepatitis C with viral replication and demonstration of hepatitis C virus-RNA in the cryoprecipitates, diagnostic of chronic hepatitis C with mixed type II cryoglobulinaemia. Treatment was begun with prednisone, 25 mg/d orally, and cyclophosphamide, 100 mg/d orally. Once hepatitis C had been diagnosed, treatment was continued with interferon alpha-2b (initially 3 x 5 mill. IU, later 3 x 3 mill. IU, each weekly subcutaneously). The vasculitis quickly healed and the cryoglobulinaemia had disappeared within 5 weeks. So far, during the treatment of 9 months, the vasculitis has not recurred, but cryoglobulins have again been demonstrated. The signs and symptoms of hepatitis C were not affected by the interferon treatment.

Two cases of the severe form of meningococcal infection are described. A 17-year-old girl and a (unrelated) 2-year-old boy suddenly developed fever and rigor. Several hours later petechiae of the skin were noted: they rapidly spread. On admission the girl was found to have a severe consumptive coagulopathy (prothrombin 24%, partial thromboplastin time 104 sec, fibrinogen 73 mg/dl, platelets 35,000/microliters). She died two-and-a-half hours after admission of treatment-resistant shock. The boy had at first only a low prothrombin value (39%), but later the other coagulation values also became abnormal. He died 16 hours after admission from the consumptive coagulopathy and profound anaemia (haemoglobin 7.4 g/dl, haematocrit 0.23). Neither patient had any clinical signs of meningitis. Isolation of Neisseria meningitidis from blood cultures confirmed the diagnosis.