"Double hit" lymphoma or secondary MYC translocation lymphoma?

Chromosomal translocations that juxtapose different genes required for proliferation and differentiation are frequently associatedwith hematologic neoplasms. A term "double hit" lymphoma refers to a group of mature B-cell malignancies that harbor MYC rearrangement accompanied with another translocation commonly found in lymphomas (i.e. IGH/BCL2). A complex karyotype with multiple abnormalities and very aggressive course of disease are additional characteristics of this group. The response to currently available treatment regimens is unsatisfying, thus reported overall survival of these patients is usually very short. Today, the precise mechanisms of "double hit" lymphoma development are still unclear, although several possible pathways have been proposed in the literature. Similar oncogenic chain of events was also observed in another common hematological malignant neoplasm – multiple myeloma. MYC translocation as a secondary pathogenic phenomenon has been demonstrated in multiple myeloma, as well as complex cytogenetics and very aggressive course of the disease. Therefore, secondary translocation involving MYC gene might be a potential marker of aggressive neoplasms emerging from different cells of origin, and united under the umbrella of "MYC-plus" malignancies. That concept might, in the future, result in a novel therapy, targeting this distinct, but not unique cytogenetic aberration

Expression and prognostic value of putative cancer stem cell markers CD117 and CD15 in choroidal and ciliary body melanoma

AIMS: The aim of the present study was to immunohistochemically investigate the expression and prognostic significance of putative cancer stem cell markers CD117 (c-kit), CD34, CD20 and CD15 in a cohort of patients with primary choroidal and ciliary body melanoma. ----- METHODS: The immunohistochemical expression of these markers was evaluated using 3,3'-diaminobenzidine tetrahydrochloride (DAB) and 3-amino-9-ethylcarbazole (AEC) chromogens on paraffin-embedded tissue samples from 40 patients who underwent enucleation in the period from 1985 through 2000. Thirty-one patients had adequate tissue specimens for the analysis. ----- RESULTS: CD117 overexpression was observed in 12 of the 31 samples (39%) when AEC chromogen was used and in 14 of 26 (54%) samples when DAB was used. CD15 positivity was seen in three out of 30 (10%) samples with AEC and in six out of 26 (23%) samples with DAB. CD20 and CD34 exhibited no positivity in the tested samples. During the average follow-up time of 8.7 years (range 0.5-22 years), 17 patients (55%) died due to metastatic disease. The Kaplan-Meier plots showed a significantly shorter overall and disease-free survival in CD117-positive patients when the AEC chromogen was used. CD15 expression was not associated with patients' survival. In multivariate analysis, patients expressing the CD117 AEC had 4.13 times higher risk of lethal outcome in comparison with CD117 AEC negative patients. ----- CONCLUSIONS: Our retrospective cohort study has for the first time demonstrated a small proportion of CD15-positive uveal melanomas. CD117 AEC overexpression was associated with a worse outcome in patients with choroidal and ciliary body melanoma. Further studies should confirm the validity of these observations and their potential for targeted treatment modalities

Erdheim-Chester Disease and Concomitant Tuberculosis Successfully Treated with Chemotherapy and Long-Term Steroids

Erdheim-Chester disease (ECD) is a rare histiocytosis usually affecting the skeletal system, but visceral organs and central nervous system involvement are common as well. Probability exists that immunomodulatory therapies and disorders can play a role in clinical course of the disease. Because of rarity of the disorder, it is hard to classify it and standardize the treatment options, but, according to published material and our experience, cytotoxic chemotherapy and long-term steroids have therapeutic benefit. Although this approach can probably be accepted as standard of care management, novel therapeutic modalities should be explored, and pathogenesis and disorder classification should be cleared out as well. The case of ECD affecting skeletal system and lungs and concomitant laryngeal tuberculosis successfully treated with chemotherapy and long-term steroid therapy is presented

Simultana pojava oralnog i kožnog anaplastičnog limfoma velikih T stanica

We present case of oral and skin anaplastic T-cell lymphoma in a 68-year-old woman. The patient presented with extensive ulcerations and necrotic tissue on the left mandibular gingiva. Orthopantomogram finding showed extensive necrolytic lesions of the adjacent mandible. Biopsy finding of oral lesions and subsequently of the skin confirmed the diagnosis of anaplastic T-cell lymphoma. The bridge on the teeth 35-37 was taken out. After three cycles of chemotherapy, oral lesions subsided, unlike skin lesions. Dentists should be aware that differential diagnosis when dealing with oral ulcerations might be the result of certain malignant hematologic diseases.U radu se prikazuje slučaj oralnog i kožnog anaplastičnog limfoma velikih T stanica u 68-godišnje osobe. U bolesnice su na prvom pregledu uočene opsežne ulceracije i nekrotično tkivo u području gingive na mandibuli lijeve strane lica. Na ortopantomogramu su se vidjele opsežne nekrolitične lezije na kosti mandibule toga dijela. Patohistološki nalaz sluznice usne šupljine, a poslije i kože potvrdio je dijagnozu anaplastičnog limfoma velikih T stanica. Most na zubima 35-37 je izvađen. Nakon tri ciklusa kemoterapije oralne lezije su se povukle, za razliku od kožnih lezija. Stomatolozi pri diferencijalnoj dijagnostici oralnih ulceracija moraju imati na umu i moguću povezanost tih lezija s malignim hematološkim bolestima