Lymphangioleiomyomatosis (LAM) causing chylothorax]

A 28-year-old woman was admitted to our hospital complaining of chest pain and dyspnea. Chest radiographs showed left pleural effusion. The effused fluid obtained by thoracocentesis was milky, and so chylothorax was diagnosed. A high-resolution chest CT (HRCT) scan demonstrated diffuse multiple cystic lesions, which were undetectable by conventional CT. An abdominal CT scan showed a retroperitoneal tumor. Since the effusion was resistant to conservative therapy, we performed clipping of the thoracic duct under the diaphragm. Since the effusion disappeared after continuous aspiration, 10 KE of OK-432 was administered into the pleural cavity, and the chylorrhea disappeared. The clinical diagnosis, based on the biopsy of the abdominal tumor, was lymphangioleiomyomatosis. Chylothorax developing from lymphangioleiomyomatosis is rare in Japan. However, we must consider the possibility of lymphangioleiomyomatosis in patients with chylothorax, and always perform chest HRCT.     

Pulmonary lymphangiomyomatosis

Lymphangiomyomatosis is an interesting disease with distinctive clinical and histopathologic findings. We report herein two additional cases of lymphangiomyomatosis, including one with clinical improvement after therapy with progesterone. This case is of particular significance in view of the patient's negative sex steroid receptor analysis. These findings open new avenues for future considerations in the therapy of this unusual but interesting disease.     

Pulmonary lymphangiomyomatosis

Pulmonary lymphangiomyomatosis is a disease of young females, who typically present with progressive dyspnea, hemoptysis, cough, repeated spontaneous pneumothoraces, and chylous effusions. The disease should be suspected when these findings are associated with an obstructive ventilatory impairment and disproportionately abnormal gas exchange. Symptoms may occur in the presence of radiographically normal-appearing pulmonary parenchyma. As the disease progresses, diffuse, prominent interstitial markings, suggestive of pulmonary fibrosis, are seen radiographically. The diagnosis is made by open lung biopsy.     

Pulmonary lymphangiomyomatosis. Demonstration of smooth muscle antigens by immunofluorescence technique.

A case is reported of pulmonary lymphangiomyomatosis in 44-year-old woman presenting with progressive exertional dyspnoea, unproductive cough and haemoptysis. The patient showed no symptoms or signs of tuberous sclerosis, a condition often found in association with pulmonary lymphangiomyomatosis. A pulmonary biopsy specimen showed disruption of alveolar septa and fibrosis. Subpleural lymph vessels and pulmonary venules showed cuffs of spindle-shaped cells. The leiomyomatous nature of these cells was evident from their appearance and staining reactions with light microscopy, and the presence of smooth muscle antigens as demonstrated by immunofluorescence technique. Since the lesion is not restricted to lymphatics, but can also be present in blood vessels, the condition may more appropriately be designated as pulmonary angiomyomatosis.     

Pulmonary lymphangiomyomatosis associated with tuberous sclerosis. Treatment with tamoxifen and tetracycline-pleurodesis

A 44-year-old woman was seen with the clinical and histologic picture of lymphangiomyomatosis syndrome. She also had dermatologic and neurologic stigmata of tuberous sclerosis. After the development of a recurring chylothorax, she had a downhill course unresponsive to dietary, bronchodilator, corticosteroid and progesterone therapy. In an open lung specimen, the search for steroid receptor for estrogen was positive. The discovery in this case of an estrogen receptor represents important evidence for establishing an association between tuberous sclerosis and lymphangiomyomatosis. Tamoxifen therapy and tetracycline pleurodesis were successful in stopping the progressive course and controlling the chylothorax.     

Pulmonary eosinophilic vasculitis in a neonate with congenital chylothorax

We report a case of congenital bilateral chylothorax, complicated by pulmonary eosinophilic vasculitis and transient eosinophilia. Excluding all known forms of eosinophilic pulmonary disease either histologically or clinically, possible mechanisms of its etiology are discussed. Treatment with corticosteroids was successful, and after a prolonged clinical course the patient recovered by age 2 years.     

Lymphangioleiomyomatosis (LAM): a rare cause of ascites and pleural effusion

HISTORY AND CLINICAL FINDINGS: A 45 year old woman presented at our hospital for further evaluation of intermittent abdominal pain. The patient reported that she had suffered a spontaneous pneumothorax 8 years and a pleural effusion 3 years before. DIAGNOSTIC FINDINGS AND THERAPY: Abdominal ultrasound showed abdominal fluid which proved to be chylous ascites by diagnostic paracentesis. Radiologic (lymphangiography) findings were consistent with lymphangioleiomyomatosis (LAM). Remarkably, lymphangiography resulted in an immediate disappearance of the ascitic fluid. CONCLUSION: This report depicts a case of LAM which involved both pulmonary and intestinal symptoms. Previous pulmonary problems gave a hint towards the right diagnosis. Though lymphangiography is rarely performed in these days, the method was both diagnostic and therapeutic in the case presented.     

Pulmonary lymphangiomyomatosis. Follow-up and long-term outcome with antiestrogen therapy; a report of eight cases.

Lymphangiomyomatosis is a rare disease which affects young women of childbearing age. Eight women with pulmonary LAM were treated with antiestrogen therapy and were monitored by blood estrogen measurements along with clinical hypoestrogenic symptoms. Treatment ranged from three to nine years. The response to therapy was evaluated by the clinical course, chest x-ray films, pulmonary function tests and overall long-term outcome. Three patients died of respiratory failure after three, five and nine years of treatment. Of the five patients remaining alive, respiratory function deteriorated in four cases, after a transient period of mild improvement lasting three years in two cases. The last patient appeared stable after three years of follow-up. Time course ranged from 4 to 17 years. However, without a control group, we cannot determine whether or not the apparent improvement of the natural time course was due to the hormonal treatment.     

Tenofovir (TDF) has stronger antiviral effect than adefovir (ADV) against lamivudine (LAM)-resistant hepatitis B virus (HBV)

Objectives We retrospectively compared the antiviral effect of tenofovir disoproxil fumarate (TDF) with that of adefovir dipivoxil (ADV) for patients with chronic hepatitis B (CHB) who developed resistance to lamivudine (LAM). Materials and methods One hundred nine patients (86 males), all Asian-American except 1 Caucasian male, with LAM resistance received TDF or ADV. HBV DNA levels were measured every 3 months. The HBeAg loss and ALT normalization were assessed at 12 months on therapy. Results Forty-four patients (37 males) received TDF (12 with LAM) and 65 (49 males) received ADV (18 with LAM). Median ages (years) for TDF and ADV were 49 (32–68) and 45 (22–68), respectively. Median duration of therapy was 13 months (6–38) and 17 months (6–34) for the TDF and ADV groups. Baseline HBV DNA levels (log₁₀ copies/ml) were 6.2 ± 1.7 for the TDF and 6.5 ± 1.6 for ADV groups. Baseline ALT (IU/l) levels were 77.0 ± 86.0 and 100 ± 195 for the TDF and ADV (P = 0.46) groups, respectively. At 12 months, mean levels of log₁₀ HBV DNA were 1.5 ± 1.0 and 4.3 ± 2.2 for TDF and ADV (P = 0.01). HBeAg loss and ALT normalization at 12 months showed no differences. Using a single factor, ANOVA (2-tailed P value), 4 groups, TDF (n = 32), TDF + LAM (12), ADV (47), and ADV + LAM (18), were compared. HBV DNA reduction at 12 months was the greatest for TDF + LAM (P < 0.001). Conclusions Our results suggest that for LAM-resistant HBV, TDF, alone or combined with LAM exerts greater viral reduction than ADV. However, no difference in HBeAg loss was observed. It appears that stronger HBV DNA reduction may not necessarily accelerate HBeAg loss. The author (H.W.H.) who has taken part in this study has declared a relationship with the manufacturers of the drugs involved either in the past or present.     

Patients' perceptions versus medical testing of function in women with lymphangioleiomyomatosis (LAM)

Lymphangioleiomyomatosis (LAM), a rare pulmonary disease that occurs primarily in women is characterized by progressive cystic lung lesions causing respiratory failure, which may require lung transplantation. It has been observed that in diseases of the lungs, objective medical tests frequently do not translate to subjective functional status. However, patient's perceptions of functional status directly impact quality of life. In order to evaluate this relationship in LAM, a cross sectional study of 31 lung transplant candidates and 43 lung transplant recipients with LAM was conducted to evaluate functional status. Objective tests (Pulmonary function tests, PFTs, 6 min walk) were obtained in 19 candidates and 23 recipients, and subjective function was assessed in all women using the Functional Performance Inventory. For both groups statistically significant relationships emerged between forced vital capacity with subjective measures of function including total, social and recreational function (P < 0.05). As well diffusing capacity related significantly to total function, ability to maintain household chores, body care, and social function (P < 0.05), where higher scores were associated with better subjective function. Forced expiratory volume in one second was not found to significantly relate to subjective function in these groups, except the physical exercise domain in transplant recipients. However, despite better PFT results in double lung transplant recipients, no differences were found in subjective measures of function between single and double lung recipients. Results suggest that clinicians need to better recognize that certain aspects of spirometry/6 min walk tests do not necessarily correlate well with patient's perceptions of their function. This has implications for the assessment and follow-up of women living with LAM; considering subjective aspects of function may help focus interventions and improve quality of life.