Diagnosis of a pericardial cyst can also be made before birth using ultrasound.The differential diagnosis for a suspected pericardial cyst includes a bronchial cyst, pericardial effusion, teratoma, lymphangioma, pericardial fat, congenital diaphragmatic hernia, neuroenteric cyst, and congenital cyst from the primitive foregut. A pericardial cyst also may look similar on imaging to a pericardial diverticula, which is connected to the pericardium and not fully walled off like a pericardial cyst. Management
Pericardial cysts that are small and cause no symptoms can be followed with repeat imaging through echocardiogram, CT, or MRI to assess for changes. Pericardial cysts that are large or cause symptoms may be candidates for percutaneous aspiration, ethanol sclerosis, or surgery. Percutaneous aspiration removes the fluid from inside the cyst, and ethanol sclerosis uses the injection of ethanol into the cyst after aspiration to decrease the likelihood of cyst recurrence. Percutaneous aspiration with ethanol sclerosis is typically the first treatment for pericardial cyst that requires management. Surgery can remove a pericardial cyst through a thoracotomy, sternotomy, mediastinoscopy, or video-assisted thoracic surgery. The type of surgery is chosen based on the size and location of the pericardial cyst. Prognosis
With most pericardial cysts having no symptoms, the prognosis is very good. The cysts can resolve without intervention. If percutaneous aspiration is performed, the recurrence rate of pericardial cysts is about 30-33%. Although pericardial cysts are benign, the location of cysts can rarely cause life-threatening emergencies by compressing the heart or lungs. Epidemiology
Pericardial cysts have an incidence of 1 in 100,000.
Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm that features a persistent neutrophilia in peripheral blood, myeloid hyperplasia in bone marrow, hepatosplenomegaly, and the absence of the Philadelphia chromosome or a BCR/ABL fusion gene. Signs and symptoms
The most common clinical finding is hepatosplenomegaly. Pruritus, gout, and mucocutaneous bleeding are occasionally seen. Cause
The cause of CNL is currently unknown. An association between CNL and multiple myeloma has been suggested based on the observation of myeloma in 20% of CNL cases. However, a clonal genetic abnormality has not been detected in these myeloma-associated cases of CNL, raising the possibility that the neutrophilia is a reaction due to the neoplastic myeloma cells. The postulated cell of origin is a limited-potential, marrow-derived stem cell. Genetics
The majority (90%) of cases have not had detectable cytogenetic abnormalities. Most importantly, the Philadelphia chromosome and other BCR/ABL fusion genes are not detected. Diagnosis
Laboratory findings
Peripheral blood neutrophilia (> 25 x 109/L) with myeloid precursors (promyelocytes, myelocytes, metamyelocytes) comprising less than 5% of leukocytes. Sites of involvement
Peripheral blood, bone marrow, spleen, and liver are most common, but any organ or tissue can be infiltrated by neutrophils. Bone marrow biopsy
On both the bone marrow aspirate and the core biopsy, a hypercellular marrow with an increased myeloid:erythroid ratio of 20:1 or greater. Myelocytes and neutrophils are increased, and blasts and promyelocytes are not increased. Due to the myeloproliferative nature of the disease, an increase in megakaryocytes and erythroid precursors may be observed, but dyspoiesis in not seen in any cell lineage. Also, reticulin fibrosis is rare.