Lymphoplasmacytic lymphoma presenting as malignant ascites.
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Biomedical subjects
Publications and source records attributed to John Lazarchick.
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Extra-adrenal myelolipomas are rare, generally solitary, non-functioning tumors consisting of adipose tissue and hematopoetic elements. These lesions are not known to be associated with underlying hematologic disorders or documented bone marrow abnormalities. We describe the extremely rare occurrence of multifocal, extra-adrenal myelolipomas in a 35-yr-old male. A markedly hypocellular bone marrow is noted, despite normal peripheral blood counts. To our knowledge, this is the first case report of a markedly abnormal marrow examination in a patient with extra-adrenal myelolipoma, and implicates the extra-osseous masses as the driving force behind his normal hematopoesis.
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PURPOSE: We describe a patient with an orbital lymphoma in which genetic analysis utilizing fluorescent in situ hybridization (FISH) on a touch preparation of the tumor identified the classic 11:14 translocation associated with a mantle cell lymphoma. DESIGN: Clinicopathologic case report. METHODS: A 76-year-old woman presented complaining of ptosis and was found to have an orbital lesion suspected of being a lymphoproliferative malignancy. A biopsy of the lesion was performed in the office, and the sample was processed using a touch preparation technique. RESULTS: Genetic analysis utilizing FISH on a touch preparation of the tumor identified the classic 11:14 translocation associated with a mantle cell lymphoma. CONCLUSIONS: FISH has become useful in the differential diagnosis of lymphoproliferative lesions. The touch preparation method requires smaller amounts of tissue than standard methods, and samples may be obtained in an office setting.
We report a 50-year-old patient with idiopathic hypereosinophilic syndrome with trisomy 8 who experienced a complete and durable hematological and cytogenetic remission with low-dose imatinib therapy. He also had a significant reversal of cardiac dysfunction with a reduction in cardiac hypertrophy, resolution of pericardial effusion and mitral and tricuspid regurgitation. He remained in remission 3 years after therapy.
HIV-related non-Hodgkin lymphoma is well documented in the literature. We report a case of an HIV-infected patient who presents with primary mediastinal large B-cell lymphoma. On review of the literature, this appears to be the first documented case of this subtype of large B-cell non-Hodgkin lymphoma seen in an HIV-infected patient. Our patient received CHOP (cyclophosphamide, hydroxydaunomycin, Oncovin, prednisone) chemotherapy with granulocyte colony-stimulating factor support but unfortunately died a few days later.
Heparin-induced thrombocytopenia (HIT) type II is an immunologically mediated reduction in platelets that increases the risk of arterial or venous thrombosis. It has been reported in up to 5% of patients receiving unfractionated heparin. Unlike other thrombocytopenic coagulopathies, HIT is associated with a high risk of thromboembolic events if not treated with an appropriate anticoagulant alternative. Diagnosis is dependent on assessment of platelet reduction, identification of previous heparin exposure, detection of thrombotic complications and evaluation of laboratory assays. HIT has been well described in surgical patient populations; however, the abdominal organ transplant population is an exception. HIT should be included in the differential diagnosis of patients presenting with thrombocytopenia after transplantation in order to prevent or treat thrombotic complications that can pose a risk to patient or graft survival.
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Sickle-beta(+) (beta(+)) thalassemia is a double heterozygous genetic disorder characterized by both a qualitative and quantitative abnormality. We present a case of an African American male who was first diagnosed with sickle cell disease (SCD) at the age 23 years when he presented with generalized bone pain, fever, and hepatosplenomegaly. Laboratory findings included thrombocytopenia, microcytic anemia, and markedly elevated ferritin. He was subsequently diagnosed with a sickle-beta thalassemia hemoglobinopathy. Findings in the bone marrow aspirate and biopsy were consistent with hemophagocytic lymphohistiocytosis (HLH). HLH resolved with the resolution of sickle cell bone pain crisis without use of immunosuppressive therapy. To the best of our knowledge this is the first documented case of HLH associated with sickle cell bone pain crisis.
A 9-month-old infant presented with hypercalcemia and lytic bone lesions. Suspicion for malignancy led to a bone marrow examination, which showed replacement of the marrow by a small round blue cell infiltrate. Flow cytometric analysis of these cells showed an unusual immunophenotype in that these cells were dim CD45, HLA-DR, and CD10 positive, but CD19, CD20, CD79a, and CD34 negative. Southern blotting showed clonal rearrangement of immunoglobulin heavy chain (IgH) which confirmed a diagnosis of precursor B acute lymphoblastic leukemia (ALL). He received supportive treatment with hydration and pamidronate, but had recurrent episodes of hypercalcemia. Once the correct diagnosis of ALL was established, the patient was treated with an infantile ALL chemotherapeutic regimen and the hypercalcemia resolved. This case highlights the usefulness of immunoglobulin gene rearrangement studies in atypical cases of ALL.
The risk of hemorrhage in infants with severe coagulopathies unresponsive to fresh frozen plasma (FFP) infusions may preclude therapeutic invasive interventional procedures. We describe the successful use of recombinant factor VIIa (rFVIIa) in two such infants, the first with cirrhosis requiring paracentesis and the second with necrotizing enterocolitis requiring laparotomy. This report reviews the current concepts on the mechanism of action of the drug rFVIIa and considers its expanded use in infants unresponsive to FFP replacement.
This study was designed to examine the relationship of short activated partial thromboplastin time (aPTT) and prothrombin time (PT) to the incidence of thromboembolic events, hereditary and acquired coagulation defects associated with an increased risk of thrombosis, or cardiovascular diseases in patients undergoing renal transplantation. The prevalence of these conditions in our patients (n = 436) was 55%. Forty-two percent of the patients had short aPTT or PT. Multivariate analysis revealed that patients with short aPTT have an odds ratio (OR) = 2.15, 95% Confidence Interval (CI) (1.27-3.64) (p = 0.0042), and for patients with short PT, an OR = 2.01, 95% CI (0.99-4.08) (p = 0.052). Our study also suggests that other risk factors, including non-white ethnicity (98% blacks), OR = 1.64, 95% CI (1.01-2.67) (p = 0.047), diabetes mellitus, OR = 2.62, 95% CI (1.11-6.18) (P = 0.028), and autosomal dominant polycystic kidney disease (ADPKD) (p < 0.0001). Short aPTT results, or probably short PT results, pre- or post-transplantation may be associated with increased risks for thromboembolism.
We present the case of a newborn male with D-transposition of the great arteries, ventricular septal defect, and subpulmonary stenosis who experienced two episodes of systemic-to-pulmonary artery shunt thrombosis. Hematologic evaluation revealed heterozygous factor V Leiden mutation. This report emphasizes the importance of evaluation for inherited coagulation disorders in pediatric patients with unexpected thrombotic complications.
Post-transplant lymphoproliferative disorders (PTLD) are a frequently fatal complication occurring after transplant. The majority of cases are of B-cell origin but a smaller percentage of cases are of T-cell origin. The etiology of these disorders is unclear, however, EBV (Epstein-Barr virus) in the context of immunosuppression is thought to play a role, particularly in B-cell PTLD. We report a case of T-cell PTLD limited to the bone marrow occurring eight years post-cardiac transplantation and review the literature on T-cell PTLD after heart transplant.
Extracellular, PAS-positive material has been described in various lymphoproliferative disorders, but is rare in malignant lymphomas. We report two such cases of grade 2 follicular lymphoma that were investigated by routine H&E histology, immunohistochemistry, flow cytometry, and molecular pathology. Ancillary studies confirmed the monoclonality in each case and the antigenic similarity to the neoplastic cells, with the exception of bcL-2. One possible pathogenic mechanism for this extracellular material may be the exocytosis of overproduced cell membranes.