A case of life-threatening refractory polychondritis successfully treated with combined intensive immunosuppressive therapy with methotrexate

Relapsing polychondritis (RP) is a rare disease of unknown etiology causing systematic inflammation and resulting in destruction of cartilaginous tissues. We describe here an 18-year-old Japanese woman who developed severe airway stenosis as the initial symptom with auricular, nasal, and ocular inflammation. The effect of high dose oral steroid, methylprednisolone pulse therapy, and cyclophosphamide was temporary and her conditioning was worsening. Finally we added methotrexate to the immunosuppressive treatment and achieved reduction of disease activity. This case illustrates the potentially fatal sudden onset of airway inflammation that can occur with this disorder, and the effectiveness of methotrexate.     

A case of chronic relapsing ANCA-associated microscopic polyangiitis successfully treated with plasma exchange

We report the case of a 4.5-year-old girl with microscopic polyangiitis (MPA) manifesting antineutrophil cytoplasmic autoantibody (ANCA)-positive necrotizing crescentic glomerulonephritis and pulmonary hemorrhage. She was initially on induction therapy with corticosteroids and azathioprine. Plasma exchange (PE) combined with immunosuppressants was used to treat an episode of recurrent pulmonary hemorrhage, and achieved remission. At 9.8 years of age her kidney disease relapsed, associated with renal dysfunction and increased proteinuria. To minimize the toxic effects of immunosuppressants, she was treated with PE again, and her renal dysfunction resolved. Plasma exchange was effective in reducing the risk of death and preserving long-term renal function without the severe adverse effects of immunosuppressants. Our preliminary results indicate that PE is likely to be a treatment option for children in acute phase of ANCA-associated MPA, who should be protected from the toxic effects of immunosuppressants.     

A case of Crimean-Congo haemorrhagic fever successfully treated with therapeutic plasma exchange and ribavirin

Crimean-Congo haemorrhagic fever (CCHF) has become an important problem in certain parts of the world. Cytokine storm plays a critical role in the pathogenesis of CCHF. Early diagnosis, supportive therapy and invasive monitoring are the cornerstones of the management of CCHF. Ribavirin is active against CCHF virus in vitro, however, evidence of clinical efficacy is still conflicting. Here, we describe a patient successfully recovered from CCHF with a combination of plasma exchange and ribavirin therapy.     

A case of acquired hemophilia caused by factor VIII inhibitor with rheumatoid arthritis,successfully treated with immunosuppressive treatment and recombinant activated factor VII

Abstract

We report the case of a patient who presented with acquired hemophilia associated with rheumatoid arthritis. The patient's factor VIII activity was less than 1% and factor VIII inhibitor was detected. Based on the blood analysis, the patient was diagnosed as having the factor VIII inhibitor. She was successfully treated with prednisolone, cyclophosphamide, and gammaglobulin to suppress the factor VIII inhibitor, and the administration of recombinant activated factor VII was effective in controlling severe bleeding episodes.     

Retinopathy in juvenile systemic lupus erythematosus successfully treated with plasma exchange

We report the case of an 11-year-old girl with juvenile systemic lupus erythematosus (SLE) and severe retinopathy. Her retinopathy was discovered during high SLE disease activity that included neuropsychiatric complications. She was successfully treated with aggressive immunosuppressive therapies and plasma exchange.     

A case of acquired hemophilia caused by factor VIII inhibitor with rheumatoid arthritis,successfully treated with immunosuppressive treatment and recombinant activated factor VII

We report the case of a patient who presented with acquired hemophilia associated with rheumatoid arthritis. The patient's factor VIII activity was less than 1% and factor VIII inhibitor was detected. Based on the blood analysis, the patient was diagnosed as having the factor VIII inhibitor. She was successfully treated with prednisolone, cyclophosphamide, and gammaglobulin to suppress the factor VIII inhibitor, and the administration of recombinant activated factor VII was effective in controlling severe bleeding episodes.     

Acquired hemophilia A

Acquired hemophilia A is a rare but severe autoimmune bleeding disorder, resulting from the presence of autoantibodies directed against clotting factor VIII. The etiology of the disorder remains obscure, although approximately half of all cases are associated with other underlying conditions. A prompt diagnosis and appropriate management enable effective control of this acquired hemorrhagic disorder: the aims of therapy are to terminate the acute bleeding episode and eliminate or reduce the inhibitor. The recent availability of bypassing agents, first activated prothrombin complex concentrates and then recombinant activated factor VII, has significantly reduced mortality during the acute phase of the disease in patients with high titer inhibitors. On another front, immunosuppressive therapy (corticosteroids and cytotoxic agents, alone or in various combinations) has resulted in long-term inhibitor suppression in up to 70% of the cases. Moreover, new therapeutic strategies (anti-CD20 monoclonal antibody and immune tolerance protocols) are very promising and may further improve the prognosis of acquired hemophilia A.     

Severe acquired hemophilia A successfully treated with activated recombinant human factor VII

 A case of acquired hemophilia A in a 65-year-old woman is presented. The patient had been subjected to cholecystectomy 2 months before the bleeding tendency appeared. On admission, she had easy bruising and prolonged activated partial thromboplastin time, but during hospitalization she had severe hemorrhage into the right gluteal and femoral muscles. An inhibitor of the factor VIII coagulant protein (FVIII : C) of high Bethesda titer was found in her serum. The patient was successfully treated with activated recombinant human factor VII (rhFVIIa) and immunosuppression. We conclude that rhFVIIa is a safe, effective, and fast-acting preparation for the treatment of severe hemorrhage in patients with acquired hemophilia A, and that the simultaneous administration of azathioprine and corticosteroids may suppress production of the inhibitor. Received: 10 February 1998 / Accepted: 9 April 1998     

Acquired chronic pure red cell aplasia successfully treated with intravenous pulse methylprednisolone therapy.

A 65-year-old male patient developed acquired chronic pure red cell aplasia (PRCA) associated with hypergammaglobulinemia and positive Coombs' test during the treatment of eosinophilic pneumonia with prednisolone (PSL). His PRCA was treated with oral PSL at a dose of 60 mg/day for 3 weeks, but anemia further progressed. Immediately after high-dose intravenous pulse methylprednisolone therapy (1 g/day for 3 days) however, reticulocyte crisis occurred and his anemia rapidly improved. He has been in complete remission under a maintenance dose of PSL for more than 2 years. This patient indicates that high-dose intravenous methylprednisolone therapy is one of the useful treatments, not only for constitutional PRCA, but also for acquired chronic PRCA.     

Nonsecretory primary plasma cell leukemia successfully treated with VAD and MP therapy

A 78-year-old woman was admitted to our hospital because of lumbago and appetite loss. Blood analysis revealed anemia, hypercalcemia and circulating plasma cells. Bone marrow aspiration showed an elevated ratio (43%) of plasma cells, which expressed CD38 in the absence of CD19 and CD56 expression. Spinal MR imaging revealed multiple compression fractures and suggested diffuse invasion of plasma cells into the spinal bodies. No M-protein was detectable in serum or urine by immunoelectrophoresis and immunofixation, but cytoplasmic M-protein (IgG-kappa) was detected by enzyme antibody staining. On the basis of the history and data, nonsecretory primary plasma cell leukemia was diagnosed. First, the patient was given modified VAD therapy (vincristine, doxorubicin, and prednisolone) and complete remission was obtained. Then MP therapy (melphalan and prednisolone) was instituted, and remission has since been maintained for 11 months. Like many other cases of primary plasma cell leukemia, this case suggests that CD56 may act as an adhesion molecule between neoplastic plasma cells and bone marrow stromal cells. Our experience with this exceedingly rare case suggests the superiority of combination chemotherapy as an induction therapy and the effectiveness of MP therapy as maintenance therapy for this disease.     

Acquired hemophilia masked by warfarin therapy

People without hemophilia but with autoantibodies specifically directed against the procoagulant activity of factor VIII are known to have acquired hemophilia. The bleeding diathesis in these patients is often severe and life-threatening. The definite laboratory diagnosis of this disorder includes demonstration of low factor VIII levels in plasma with a high titer of factor VIII inhibitors, but the initial suspicion for its presence should rise in view of a prolonged partial thromboblastin time (PTT) and a normal prothrombin time associated with an acquired bleeding disorder. Oral anticoagulant treatment is known to prolong PTT as well, and the merger of these 2 situations may cause delayed diagnosis of acquired hemophilia with devastating consequences. We describe here the first reported case of acquired hemophilia diagnosed in a patient treated with warfarin. In such patients prolonged PTT may be ascribed to warfarin therapy rather than to acquired hemophilia, thus causing a dangerous delay in diagnosis.     

A second attack of cholestasis associated with erythropoietic protoporphyria was successfully treated by plasma exchange and blood transfusion

Erythropoietic protoporphyria (EPP) is a rare hereditary disease that can sometimes cause acute liver failure based on cholestasis. Acute liver failure is a fatal complication and is associated with EPP in 1–4 % of patients. Although it is extremely difficult to recover from acute liver failure, we experienced an important case of EPP where the patient recovered from the first attack of cholestasis with antibiotic treatment. The patient recovered from a second attack of cholestasis with blood infusion and plasma transfusion. This case suggests that the supply of heme by blood transfusion and the elimination of excess protoporphyrin production by plasma exchange may be a useful treatment for patients with acute cholestasis associated with EPP.     

Isolated pulmonary hypertension in diffuse cutaneous systemic sclerosis successfully treated with long-term plasma exchange

Isolated pulmonary hypertension (PHT) in patients with diffuse cutaneous systemic sclerosis (dSSc) is one of the most severe complication. Here we report the case of a 22 year-old white woman with anti-U3RNP antibody-positive dSSc complicated by severe, isolated PHT successfully treated with long-term plasma exchange. This beneficial effect persisted for two years, even after plasma exchange discontinuation. This is the first observation of isolated PHT in dSSc responsive to plasma exchange therapy.     

Thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus successfully treated with plasma exchange and corticosteroids

Thrombotic thrombocytopenic purpura (TTP) is a rare dynamic life-threatening disease with systemic formation of platelet thrombi in the microvasculature within all the organs. Until recently the mortality in TTP has exceeded 90%. The progress of medical research in the last two decades has significantly improved our understanding of the pathogenesis of TTP and allowed a reduction in mortality. This paper describes a case of a 36-year-old female patient with systemic lupus erythematosus (SLE) who was admitted to our hospital for jaundice and speech disturbances. Laboratory tests revealed hemolytic anemia with a negative Coombs test. Abundant schistocytes were present in peripheral blood smear. During the first day of hospitalization progression of neurological signs was observed -the patient received high-dose corticosteroids (1000 mg of methylprednisolone daily for 5 consecutive days) and underwent plasma exchange therapy. We observed significant improvement of the patient's condition, as well as a complete resolution of clinical and laboratory abnormalities. In the following 24 months there have been no signs of the relapse of TTP. The article contains also a brief update of this unusual disease.     

A case of steroid-resistant acute hypersensitivity pneumonitis successfully treated with an immunosuppressive drug]

A 28-year-old man complaining of cough and fever was hospitalized because of bilateral diffuse granular lung shadows. Hypersensitivity pneumonitis was diagnosed based on bronchoalveolar lavage fluid (BALF) and transbronchial lung biopsy (TBLB). Since antigen avoidance alone was not effective, steroid pulse therapy was started, and his symptoms and chest X-ray findings improved. After discharge, he moved to another residence. A few weeks later, fever and dyspnea recurred, then he was hospitalized on the suspicion of acute exacerbation of hypersensitivity pneumonitis. Steroid therapy resulted in no improvement on this occasion. Lung biopsy under video-assisted thoracoscopy was performed, and acute hypersensitivity pneumonitis was diagnosed pathologically. Although steroid therapy was continued, hypoxia still remained and a KL-6 level markedly increased. Combined therapy with steroid and cyclosporin A was started, and his symptoms, physical findings, laboratory data, and chest X-ray findings gradually improved. There has been no report in which cyclosporin A was used for acute hypersensitivity pneumonitis but this case indicates that cyclosporin A is efficacious for its treatment.