Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity.

The syndrome resulting from combined pulmonary fibrosis and emphysema has not been comprehensively described. The current authors conducted a retrospective study of 61 patients with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs on chest computed tomography. Patients (all smokers) included 60 males and one female, with a mean age of 65 yrs. Dyspnoea on exertion was present in all patients. Basal crackles were found in 87% and finger clubbing in 43%. Pulmonary function tests were as follows (mean+/-sd): total lung capacity 88%+/-17, forced vital capacity (FVC) 88%+/-18, forced expiratory volume in one second (FEV1) 80%+/-21 (% predicted), FEV1/FVC 69%+/-13, carbon monoxide diffusion capacity of the lung 37%+/-16 (% predicted), carbon monoxide transfer coefficient 46%+/-19. Pulmonary hypertension was present in 47% of patients at diagnosis, and 55% during follow-up. Patients were followed for a mean of 2.1+/-2.8 yrs from diagnosis. Survival was 87.5% at 2 yrs and 54.6% at 5 yrs, with a median of 6.1 yrs. The presence of pulmonary hypertension at diagnosis was a critical determinant of prognosis. The authors hereby individualise the computer tomography-defined syndrome of combined pulmonary fibrosis and emphysema characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival.     

Combined hepatocellular-cholangiocarcinoma: A clinicopathological study

Combined hepatocellular-cholangiocarcinoma (HCC-CC) is an uncommon form of primary liver cancer having features of both hepatocellular and biliary epithelial differentiation. We reviewed 21 cases of this tumour diagnosed between 1972 and 1996 (patient age range 16–79 years; mean patient age 49.7 years; 18 male and three female patients). Histologically, the majority (n= 18) of tumours were ‘mixed’ tumours, in which areas of hepatocellular and biliary epithelial differentiation were intimately mixed within the same tumours. Two patients had separate tumours in which discrete nodules of HCC and CC occurred in the same livers. One patient had a ‘fibrolamellar’ tumour that histologically simulated the fibrolamellar variant of HCC, but some of the tumour cells were mucin-producing cells. Of the 21 cases, mucin was demonstrable in 16 and, in the few mucin-negative tumours, electron microscopic studies confirmed the presence of the dual differentiation. The tumours frequently exhibited an invasive character with frequent venous permeation, direct invasion into adjacent liver parenchyma and tumour microsatellite formation, similar to that of ordinary HCC. Histological evidence of cirrhosis or chronic hepatitis was present in 77.8% of patients and 75% of patients were hepatitis B surface antigen positive. Raised serum α-fetoprotein (AFP) levels (above 300 ng/mL) were present in 61.5% of patients and AFP was detected immunohistochemically in 55% of tumours. The overall survival times of patients with HCC-CC were short. In conclusion, HCC-CC showed clinical and pathological features more akin to those of ordinary HCC than to CC.     

Combined hepatocellular-cholangiocarcinoma associated with dermatomyositis

A 56 year old female developed combined hepatocellular cholangiocarcinoma associated with dermatomyositis. Serum tumour markers except for carbohydrate antigen (CA 19-9; 6400 ng/ml) were within normal range. Despite extensive chemotherapy, no clinical response was obtained and the patient's course deteriorated after 4 months. Macroscopically, the liver was mainly occupied by hepatocellular carcinoma but cholangiocarcinoma was found in the hilum. This is the first case of a rare association of combined hepatocellular-cholangiocarcinoma and dermatomyositis.     

Immunoglobulin D amyloidosis: a distinct entity

IgD monoclonal gammopathies are uncommon. They are seen rarely as a monoclonal gammopathy of undetermined significance and are present in 1%-2% of patients with multiple myeloma. In light-chain amyloidosis, IgD monoclonal proteins are found in ap-proximately 1% of patients. When an IgD monoclonal protein is found, amyloidosis is often omitted from the differential diagnosis. In the present study, we reviewed the natural history of IgD-associated amyloidosis among 53 patients seen over 41 years. The distribution of clinical syndromes suggests that these patients have a lower frequency of renal and cardiac involvement. The overall survival of these patients does not appear to be different from that of patients who have light-chain amyloidosis associated with another monoclonal protein.     

Sudden asphyxic asthma: a distinct entity?

This study analyzed the history, clinical characteristics, and acid-base data in relation to the speed of decompensation in 34 patients intubated and mechanically ventilated for severe asthma. Three patterns of decompensation were established according to the delay between the onset of symptoms and endotracheal intubation: Group I, rapid decompensation (less than 3 hours); Group II, gradual development of respiratory failure (9.2 +/- 7.7 days); Group III, acute exacerbation after unstable asthma (4.2 +/- 3.6 days). Patients who developed sudden asphyxia (Group I) showed features distinct from those with a gradual worsening. Sudden asphyxic asthma is more frequent in young men and is characterized by a severe mixed acidosis with extreme hypercapnia (mean PaCO2 = 112.8 +/- 43.9 mm Hg), a higher incidence of respiratory arrest, and silent chest upon admission. Recovery is more rapid, with a shorter duration of mechanical ventilation (33.7 +/- 25.3 h versus 91.4 +/- 64.1 h in Group II). Several arguments suggest that bronchospasm plays the primary role in the pathogenesis of sudden asphyxic asthma.     

Eosinophilic fasciitis: a distinct clinical entity?

Eosinophilic Fasciitis is a syndrome characterized by exertion related scleroderma-like skin changes, peripheral eosinophilia, hypergammaglobulinemia and diffuse faciitis. Controversy exists as to the precise classification of the syndrome, i.e., whether it is a distinct entity or a variant of scleroderma. We describe a patient with eosinophilic faciitis but with several unique features: 1) progressive skin changes unresponsive to corticosteroid therapy; 2) elevated anti-DNA antibodies; 3) hypocomplementemia; and 4) a followup biopsy showing sclerodermatoid skin changes. These features and others relating to the controversial aspects of classification of eosinophilic fasciitis are discussed.     

Primary pulmonary Hodgkin's disease: a distinct entity

A 30-year-old lady presented with fever, dry cough and weight loss for the preceding five months. Radiological investigations revealed a solitary nodular lesion in the lingula of the left lung. Guided fine needle aspiration cytology failed to yield any diagnostic material. Bronchoscopic cytology was also not contributory. As a last resort open lung biopsy was done and a diagnosis of Hodgkin's disease was made. Hilar and pre aortic lymph node biopsies showed only reactive change. The final diagnosis was primary pulmonary Hodgkin's disease.     

Healthcare associated infective endocarditis: a distinct entity

The terms hospital- and community-acquired infections do not cover any longer the full spectrum of acquisition of infection. Consequently, the term healthcare associated infection (HCA) has been recently introduced. In order to examine the applicability of 'HCA infection' to patients with infective endocarditis (IE), 125 episodes of culture-positive IE were categorized into 3 groups of acquisition. 14 (11%) of 125 episodes were defined as hospital acquired (HA) IE (onset of more than 72 h after admission), 52 (42%) as HCA (IE on admission in patients with significant previous healthcare contact), and 59 (47%) as community acquired (CA) (IE on admission in people without recent healthcare contact). 41 (77%) of the 53 causative agents in the HCA IE group were typical nosocomial pathogens, whereas these types of pathogens constituted only 22% (14/64) of the microorganisms in the group of CA IE (p<0.0001). Mortality in the HA and HCA groups combined was significantly higher than that in the CA group (19/62, 31%, vs 6/59, 10%, p=0.01). HCA IE should be recognized as a distinct category that constitutes a large proportion of all cases of IE. HCA IE is significantly different from CA IE and, therefore, may require a different therapeutic approach.     

Minimally differentiated acute myeloid leukemia (AML-MO): a distinct clinico-biologic entity with poor prognosis

 FAB proposals for the diagnosis of AML-M0 represent the formal recognition of a distinct entity which has been described over the past few years by several authors and called minimally differentiated acute myeloid leukemia. By definition, AML-M0 includes acute leukemias which do not fit morphological and cytochemical criteria for the diagnosis of AML, and for which myeloid lineage assignment can be made by immunological assay showing positivity for MPO, CD13, and CD33 and negativity for lymphoid markers. Involvement of an early myeloid progenitor in the leukemic process is a possible theory hypothesized to explain the existence of such a form. Validity of this assumption has been based on the observation that AML-M0 frequently bears "stem cell" markers such as CD34, HLA-DR, Tdt, CD7, and promiscuous IgH/TCR gene rearrangements, which are thought to occur in uncommitted cells. Finally, AML-M0 very frequently carries cytogenetic abnormalities common to MDS or secondary AML, such as -5/5q- or -7/7q- deletions and or complex karyotype. In our experience, AML-M0 is also very often associated with the MDR phenotype, which in turn has been found strictly linked to "stem cell" features, especially in MDS. These biological aspects, altogether, translate into a very unfavorable prognosis, confirming even from a clinical point of view that AML-M0 is a distinct entity. In conclusion, "stem cell" markers, MDR phenotype, complex chromosome lesions, frequent occurrence in elderly patients, and intrinsic chemoresistance characterize AML-M0 and indicate the need for tailored treatments, possibly involving the use of MDR modulators and/or differentiating agents. Received: 20 November 1995 / Accepted: 19 December 1995     

Portal hypertensive polyps: distinct entity

Introduction

Gastric mucosal changes in portal hypertension (PH) are well known, but gastroduodenal polyps in PH are rarely described.

Aim

This study aims to estimate prevalence of upper gastrointestinal (GI) polyps in patients with PH of any etiology and to evaluate the role of angiogenesis in portal hypertensive polyps.

Material and Methods

This is a retrospective analysis of all patients undergoing upper GI endoscopy to compare the etiology of the polyps in the portal hypertensive group vs. those without PH. The diagnosis of polyps was done using standard histological criteria. Another part of the study consisted of prospective analysis of vascular proliferative marker CD 34 and morphometry in 47 patients.

Results

A total of 3,811 upper GI endoscopies were done of which 121 patients (3.2 %) had polyps in upper GI tract. In patients with PH (=631), polyps were noted in 16, portal hypertensive polyps in 9, hyperplastic in 6, and fundic gland polyp in 1. In the patients without PH (n?=?3,180), polyps of various etiologies were noted in 105 patients. The prevalence of polyps of all causes was similar in both groups (2.5 % vs. 3.3 %, p?=?0.3957). Prevalence of hyperplastic polyps was similar in PH (0.95 %) and non-PH group (1.3 %). On immunohistochemistry, PH polyps and PH gastric mucosa had significantly higher vessel diameter of >50 μm, increased vascular density as compared to non-portal hypertensive polyps (PHP) and normal gastric mucosa.

Conclusion

PHP are definite identifiable lesion in patients of cirrhosis with PH. PHP are probably related to increased angiogenesis in gastric mucosa.     

Minimally differentiated acute nonlymphocytic leukemia: a distinct entity

Ten of 136 consecutive adult patients with previously untreated acute leukemia had morphologically undifferentiated leukemia by light microscopy. Leukemic cells from these patients were characterized by agranular cytoplasm, negative histochemical staining with sudan black (SB) and nonspecific esterase, and absent lymphoid cell surface markers and therefore were not classifiable according to the French-American-British (FAB) system. Electron microscopy with myeloperoxidase (MPO) staining revealed the presence of peroxidase positive cytoplasmic granules and endoplasmic reticulum in eight of the nine patients studied. Cells from the patient who was negative for MPO were also negative for platelet peroxidase. A series of monoclonal antibodies to myeloid antigens also revealed myeloid features with all patients having at least one myeloid differentiation antigen present on the surface of their cells. Common acute lymphoblastic leukemia (ALL) antigen was absent in the nine patients tested. Cytogenetic analysis of blast cells was abnormal in seven patients on whom adequately banded chromosomes were obtained although there were no consistent abnormalities. No patient had a Ph1 chromosome. Only two of the ten patients achieved a complete remission. Morphologically undifferentiated leukemia may have myeloid features when studied by transmission electron microscopy or with monoclonal antibodies for cell surface markers. Such studies should be performed when the leukemia cannot be classified using either light microscopy or lymphoid cell surface markers. Such patients infrequently achieve remission with standard therapy and constitute a distinct entity.     

Examination of isolated ventricular noncompaction (hypertrabeculation) as a distinct entity in adults

Three patients (2 women) 36, 45, and 49 years of age underwent cardiac transplantation for what was diagnosed clinically as nonischemic dilated cardiomyopathy. Examination of the transthoracic echocardiogram and explanted heart in each disclosed marked hypertrabeculation involving the free wall of the very dilated left ventricle, a finding consistent with what has been termed "isolated ventricular noncompaction" (IVNC). Although these 3 cases anatomically fulfilled the echocardiographic definition of IVNC, review of previous publications containing gross photographs of the heart suggests that IVNC is overdiagnosed at least morphologically.     

Combined hepatocellular-cholangiocarcinoma presented with massive pulmonary embolism

A 30-year-old HBsAg-positive woman was admitted to the hospital because of 6 days of progressive shortness of breath. She was in severe respiratory distress with circulatory collapse. She had an enlarged liver but no stigmata of chronic liver disease or signs of cirrhosis. She had rapidly developed respiratory arrest and was transferred to intensive care unit. Heart ultrasonography and Doppler scan showed right heart straining and high pulmonary artery pressure. Despite cardiovascular and respiratory support she died a few hours after admission. Autopsy revealed combined hepatocellular-cholangiocarcinoma infiltrating the entire liver, metastatic invasion of lung blood vessels and absence of right ventricular hypertrophy. The incidence of hepatocellular-cholangiocarcinoma, a variant of hepatocellular carcinoma, is roughly 2-3% and the presenting symptoms are abdominal pain, weight loss, jaundice, fever or decompensation of liver disease. Associated HBsAg positivity and cirrhosis are reported in 20-30% and 60% of patients, respectively. Metastases to lungs are relatively frequent but this is the first report of hepatocellular-cholangiocarcinoma presented with acute respiratory distress due to massive pulmonary embolism.