Still's disease associated with necrotizing lymphadenitis (Kikuchi's disease): report of 3 cases

Necrotizing lymphadenitis (Kikuchi's disease) is recognized as one of the benign lymphadenopathies. Previously, this condition has not been described in Still's disease which is frequently associated with lymphadenopathy. We report 3 cases of Still's disease, 2 in adults and 1 in an older child, with the findings of necrotizing lymphadenitis on lymph node biopsy. Considering the common infectious and/or immunologic etiologies described in both disorders, coexistence of both conditions suggests the possibility that they share a common etiology.     

Parvovirus B19-associated haemophagocytic syndrome with lymphadenopathy resembling histiocytic necrotizing lymphadenitis (Kikuchi's disease)

A 15-year-old girl developed a haemophagocytic syndrome caused by human parvovirus B19 (PVB19). The cervical lymph node histology, resembling that of histiocytic necrotizing lymphadenitis (HNL, Kikuchi's disease), included several transformed lymphocytes, numerous histiocytes, and massive necrosis. We detected PVB19-positive cells in the lymph node by immunohistochemistry. Possible autoimmune mechanisms in HNL-like diseases triggered by PVB19 are discussed.     

Parvovirus B19 infection can induce histiocytic necrotizing lymphadenitis (Kikuchi's disease) associated with systemic lupus erythematosus.

We report three cases of systemic lupus erythematosus (SLE) associated with necrotizing histiocytic lymphadenitis (Kikuchi's disease) and immunologically proven human parvovirus B19 infection. Simultaneous occurrence of SLE and Kikuchi's disease was a characteristic of the three cases. Kikuchi's disease is an uncommon disease that usually affects young women and is characterized by painless unilateral cervical lymph-node enlargement. T-cell regions of affected lymph nodes are exclusively involved with patchy paracortical necrosis surrounded by a polymorphous cell population of histiocytes and macrophages. However, lymphadenopathy in patients with SLE may be histologically indistinguishable from Kikuchi's necrotizing lymphadenitis. The cause of Kikuchi's disease remains uncertain, although infectious agents have been proposed. A positive IgM-specific anti-human parvovirus B19 antibody test in our three cases suggests that B19 can induce a necrotizing histiocytic lymphadenitis and possibly a clinical SLE flare. High-dose (1 mg/kg/day) and medium-dose (0.5 mg/kg/day) oral prednisone was an effective treatment for constitutional and visceral symptoms of Kikuchi's and SLE diseases.     

Kikuchi's necrotizing lymphadenitis: a cause of fever of unknown origin and splenomegaly

Kikuchi's disease is characterized by lymphadenopathy in young patients and may be mistaken for malignant disease both clinically and histologically. We report the case of a 26-year-old man with persistent fever for 3 weeks and splenomegaly, in whom pancytopenia developed afterwards. A bone marrow aspiration was normal. Blood, urine, throat, stool and bone marrow cultures were negative as were serological tests for lues, Toxoplasma, Epstein-Barr virus and Widal's test. An abdominal CT scan showed enlarged retroperitoneal lymph nodes and an exploratory laparotomy was performed. Two lymph nodes were excised and a wedge biopsy of the liver was performed. The histological findings in the lymph nodes were compatible with the diagnosis of Kikuchi's disease. The patient became afebrile on the 2nd postoperative day without any treatment. He has been well for 4 months after discharge.     

Elevated serum interferon γ and interleukin-6 in patients with necrotizing lymphadenitis (Kikuchi's disease)

We investigated serum levels of interferon α, interferon γ, tumour necrosis factor α, interleukin-2 (IL-2) and interleukin-6 (IL-6) in patients with necrotizing lymphadenitis (Kikuchi's disease) (NL). Four male patients, diagnosed as having NL following biopsy of the affected lymph nodes and by the clinical course, were included in this study. All four patients had higher than normal serum interferon γ and IL-6 levels during the acute phase, which returned to normal levels during the convalescent phase. Interferon α, tumour necrosis factor α and IL-2 were, however, within the normal ranges. Our findings indicate the possibility that interferon γ and IL-6 may play an important role in the pathogenesis of NL.     

Treatment of fulminant adult Still's disease with intravenous pulse methylprednisolone therapy

We describe a patient with fulminant adult onset Still's disease and multiple organ involvement. The acute flare persisted despite high doses of prednisone but responded to megadose intravenous pulse methylprednisolone therapy. This treatment modality may be useful in temporizing severe multisystem exacerbations of adult Still's disease.     

Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto's disease) mimicking systemic lupus erythematosus: a review of two cases

Kikuchi-Fujimoto's disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign and self-limited lymphadenitis commonly found in young women. It often shares clinical features with systemic lupus erythematosus (SLE), such as arthralgias, fever and leukopenia. The etiology of KFD remains unknown and controversial. Clinical course is favorable, with spontaneous remission in less than four months in almost all cases. Herein, we present two cases. The former is a 53-year old woman presenting with cervical lymphadenopathy, arthralgia, pancytopenia and positive antinuclear antibody (ANA). Lymph node biopsy revealed histopathological features compatible with Kikuchi-Fujimoto histiocytic necrotizing lymphadenitis. The latter patient was a 20-year old woman presenting with left cervical lympadenopathy, a butterfly rash that was reminiscent of SLE, and a positive antinuclear antibody. Based upon clinical, histological and laboratory findings, the diagnosis of SLE was excluded. Careful attention should be paid to differentiating between KFD and SLE, because of their similar presentations, yet different clinical courses and therapeutic requirements.     

Immunosuppressive therapy for myelodysplastic syndrome: efficacy of methylprednisolone pulse therapy with or without cyclosporin A

We investigated whether immunosuppressive therapy using methylprednisolone (mPSL) with or without cyclosporin A (CsA) could benefit patients with myelodysplastic syndrome (MDS). Eligibility criteria for this study were a clinical diagnosis of MDS with less than 5% blast in peripheral blood, less than 10% blast in bone marrow and advanced cytopenia. Among 73 patients with MDS, 18 eligible and consecutive patients (8 men and 10 women), aged 48 to 87 years (median: 66.5 years) were assigned to receive mPSL pulse therapy (1,000 mg daily for 3 consecutive days, followed by tapering oral prednisolone; n= 12) or mPSL pulse with CsA therapy (4 to 5 mg/kg administered twice daily; n= 6). Six of 18 patients (33.3%; 3 of 10 patients with RA, 2 of 6 patients with RAEB, 1 of 2 patients with CMMoL) responded to immunosuppressive therapy. Four patients responded to mPSL pulse alone, and two patients responded to mPSL pulse with CsA. One of 6 patients with hypocellular bone marrow and 5 of 12 patients with normocellular or hypercellular marrow responded to immunosuppressive therapy. No patient with myelofibrosis responded to the therapy. The duration of response ranged from 4 to 59 months (median: 14 months). Although a significant difference was observed between responders and nonresponders in the survival rate (P<0.05), no significant difference was found in clinical characteristics at entry between responders and nonresponders. In responders, mean hemoglobin levels were significantly increased (P<0.01), and the required red cell transfusion dose was significantly reduced (P<0.01). It is possible that immunosuppressive therapy might be effective for a certain subset of patients with MDS.     

Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) associated with antiphospholipid syndrome: case report and literature review

Histiocytic necrotizing lymphadenitis (HNL), or Kikuchi-Fujimoto disease, is a benign, self-limited disease that predominantly occurs in women. The etiology remains undetermined, although a viral or autoimmune hypothesis has been suggested. The disease usually emerges with cervical lymphadenopathy with or without fever. The diagnosis can be confirmed only by histological findings of lymph node biopsy, characterized by necrosis and histiocytic infiltration without neutrophils. We report a case of a 28-year-old woman with a medical history of two episodes of unexplained pulmonary embolisms (3 and 2 years previously) who was admitted to our hospital because of unilateral cervical lymphadenopathy and mild fever that presented 1 week before admission. A diagnosis of HNL was performed by lymph node biopsy. In parallel, whereas the laboratory tests for inherited thrombophilia were negative, a progressive elevated titer of anti-beta(2) glycoprotein I (GPI) antibodies was established. Because of persistent fever, the patient received a short course of corticosteroid therapy and she recovered completely from the HNL after 2 months. It is noteworthy that to date the patient has displayed an elevated titer of anti-beta(2) GPI antibodies (18 months after the recovery from the HNL). Thus, considering the previous history of venous thrombosis and the presence of antiphospholipid antibodies, the diagnosis of primary antiphospholipid syndrome associated with HNL was made. To our knowledge, this is the first report in the literature describing antiphospholipid syndrome associated with HNL. Moreover, a brief literature review is provided with emphasis on the etiology, clinical course, and pathogenesis of this rare disease entity.     

Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto's disease) accompanied by hemophagocytosis and salivary gland swelling in a patient with systemic lupus erythematosus

After 2 years of steroid therapy that had effectively controlled her systemic lupus erythematosus, a 37-year-old woman presented with fever, erythema (face, upper chest), and low CH50. Increased oral steroid (prednisolone from 15 mg to 40 mg) and intravenous methylprednisolone (mPSL) (80 mg for 3 days) alleviated these symptoms except for the fever. Subsequently, the patient's fever worsened and leukocytopenia, abnormal liver function, lymphadenopathy (neck, axilla), and salivary gland swelling developed. Lymph node histology revealed features characteristic of Kikuchi-Fujimoto's disease (KFD). Laboratory examinations showed WBC 600/microliter, Hb 9.5 g/dl, platelets 90,000/microliter, GOT 766 IU/l, GPT 646 IU/l, LDH 4,228 IU/l, TG 1,622 mg/dl, and ferritin 6,330 ng/ml. Serum interferon gamma was also elevated (673 U/ml). Because a bone marrow smear revealed hemophagocytosis, mPSL pulse therapy (1 g for 3 days) was started for treatment of hemophagocytic syndrome. The fever promptly disappeared, and the patient's clinical symptoms resolved within 2 weeks. The abnormal laboratory data related to KFD and hemophagocytosis returned to normal within 4 weeks after the initiation of mPSL pulse therapy. We speculated that the hemophagocytosis and salivary gland involvement in this patient were also symptoms of KFD. This case indicated that corticosteroid pulse therapy is effective for KFD with serious clinical symptoms.     

Treatment of renovascular hypertension by transluminal angioplasty--13 years experience in a single centre

OBJECTIVE: The study is a follow-up on treatment of renovascular hypertension (RVH) with percutaneous transluminal renal angioplasty (PTRA). METHODS: Patients were screened on the basis of clinical criteria of increased probability of RVH with renography and in selected cases with renal vein renin measurements. A positive work-up suggesting a functionally important renal artery stenosis led to renal angiography and PTRA if stenosis was confirmed; in 59%, an intravascular stent was inserted. RESULTS: Outcome of treatment was classified as follows - group I: normotensive without medication; group II: with improved control of blood pressure; group III: unchanged blood pressure control. Grouping was performed immediately after treatment, at 1 month, 6 months and at the latest follow-up. One hundred-and-twenty-two patients (124 atherosclerotic and 12 fibromuscular lesions) were treated during 13 years. Immediately after PTRA the patients were grouped as follows - I: 31%, II: 59%, III: 10%. At 1 month, I: 13%, II: 72%, III: 15%; at 6 months, I: 11%, II: 74%, III: 15%, and at the latest follow-up, I: 11%, II: 78%, III: 11%. There were few significant complications, and renal function remained on average stable. CONCLUSION: PTRA is an effective treatment of RVH in patients selected by signs of a flow-restricting stenosis. Twelve percent were normotensive after angioplasty and a further 77% had better controlled hypertension. Few complications were seen and renal function was on average unchanged as measured by serum creatinine.     

Malnutrition,inflamation and atherosclerosis (MIA syndrome) in patients with end stage renal disease on maintenance hemodialysis (a single centre experience)

Background

Inflammation and malnutrition play an important role in endothelial dysfunction, atherosclerosis and excessive cardiovascular morbidity and mortality in ESRD patients

Gastrointestinal stromal tumor (GIST) -- single center experience of prolonged treatment with imatinib

BACKGROUND: The tyrosine kinase inhibitor imatinib has been introduced into the treatment of gastrointestinal stromal tumors (GIST). Here we report our results of prolonged treatment in comparison to a similar group of GIST patients who had died before imatinib became available. METHODS: Fourteen patients with recurrent or metastatic GIST were treated with imatinib. Clinical data and tumor samples of ten patients from the pre-imatinib era were available for comparison. Comparative genomic hybridisation (CGH) was performed on tumors to identify changes that may predict response to treatment. RESULTS: Fourteen patients were treated, mean treatment time 22.3 months (1 non-response, 2 progression after initial response, 2 stable diseases, 8 partial responses, 1 complete response). Adverse side effects were mild in general. Survival was higher in the treated group (41.1 months vs. 34.8 months in the historical group). Eleven treated patients are alive. CGH analysis showed comparable numbers of chromosomal aberations in both groups. CONCLUSION: Prolonged treatment with imatinib is safe and effective in patients with recurrent or metastatic GIST.     

Idiopathic granulomatous hepatitis with a prolonged course: effect of corticosteroid therapy.

The effect of corticosteroid (or ACTH) therapy on 4 patients with idiopathic granulomatous hepatitis is described. All patients presented with spiking fever and chills and none had jaundice. Only 1 patient had an enlarged tender liver and 3 had splenomegaly. The erythrocyte sedimentation rate was increased in all cases while the white blood cell count was typically normal. Impairment in liver function was insignificant and consisted of a mild elevation of SGOT and alkaline phosphatase activities and prolonged prothrombin time. All patients presented a diagnostic challenge. The diagnosis was established by routine liver biopsies in 3 cases and by laparotomy in the 4th. The etiology could not be established. All patients reacted dramatically to prednisone (or ACTH) after failure of other therapeutic regimens. The disease has, however, been present for 5 years in 1 patient and 10 years in another, Relapses occur after cessation of therapy.     

Evaluation of intraoperative microwave coagulo-necrotic therapy (MCN) for hepatocellular carcinoma: a single center experience of 719 consecutive cases

Background

Hepatic resection (HRx) or radiofrequency ablation may be carried out as the first-line treatment of hepatocellular carcinoma (HCC). However, we have used intraoperative microwave ablation, named microwave coagulo-necrotic therapy (MCN) as part of our strategy for the treatment of HCCs for more than 15 years. Here we describe the treatment outcomes achieved at our institution as a high-volume center for microwave ablation.

Methods

Between July 1994 and December 2010, 719 consecutive patients received MCN as their initial therapy for HCC (mean tumor size 26.9 mm, mean number of lesions 2.51) at our institute. The therapeutic survival effect, local tumor progression, and overall recurrence were prospectively evaluated and statistically analyzed.

Results

The 1-, 3-, 5-, 7-, and 10-year overall survival rates of all 719 patients were 97.7, 79.8, 62.1, 45.3, and 34.1 %, respectively. Thirty percent of the patients had Child–Pugh class B cirrhosis and the 5-year survival rate of these patients was 46.6 %. The 5-year survival of patients with ≥4 lesions (n = 168) was 49.6 % and that of patients meeting the Milan criteria (n = 470) was 70.9 %. The 1-, 3-, 5-, 7-, and 10-year overall survival rates for 390 patients treated with MCN who had ≤3 lesions with diameter ≤3 cm were 97.9, 85.1, 70.0, 57.1, and 43.0 %. No significant differences were found between the overall survival rates after MCN and the overall survival rates in 34 patients treated with HRx during the same period at our institute and under the same (P = 0.3592), nor were there any differences in disease-free survival (P = 0.3496) and local recurrence rates between the MCN and HRx groups (P = 0.5926).

Conclusion

MCN is effective for the locoregional control of HCC, with results comparable to those of HRx. MCN should be considered as one of the first-choice treatments for HCC, even for patients with poor liver function or multiple lesions.     

Hemolytic uremic syndrome following prolonged gemcitabine therapy: report of four cases from a single institution

Hemolytic uremic syndrome (HUS) has been described following the administration of multiple antineoplastic agents, most notably mitomycin C. More recently, several cases of gemcitabine-induced HUS have been observed with the overall incidence of gemcitabine-induced HUS estimated at 0.015–0.25%. We here report on four patients who developed HUS following gemcitabine therapy at our institution within the last year (incidence 1.4%). All these patients had advanced-stage disease, were heavily pretreated, and received prolonged gemcitabine application, suggesting that in this subgroup of patients HUS may be more frequently encountered than documented so far.     

Low-dose MTX(methotrexate) pulse therapy in a patient with putsulotic-arthro-osteitis (PAO) accompanied with Basedow's disease

A 62-year-old woman, who had suffered from putsulosis palmaris et plantaris for about two years, was admitted to our hospital due to lumbago, sweating and palpitation. On the basis of physical and endocrinological examinations, she was diagnosed as Basedow's disease. X-ray findings revealed ossification in the left costo-clavicular joints, and characteristic syndesmophytes of PAO in vertebral bodies. These lesions were followed by high accumulation of 99mTc in bone scintigrams. PAO is considered to be in a category of seronegative spondylarthropathy or rheumatic disease. Previous reports of PAO accompanied with autoimmune diseases are few. The association of PAO and Basedow's disease has not been reported. As PAO in this case was refractory to conventional therapies, low-dose MTX pulse therapy(oral 7.5mg/day administration on every week) was initiated. MTX markedly improved both putsulotic palmaris et plantalis and PAO.