Clinical features and therapeutic outcome of 30 patients diagnosed with primary myelofibrosis at the national center of hematology

Carotid body paraganglioma is a rare neuroendocrine tumor presenting with low-grade histological and clinical features. However, the tumor has the potential to produce distant metastasis, and due to its rarity, little information is available regarding chemotherapy for such metastatic lesions. Here, we report a case of carotid body paraganglioma with development of pulmonary and bone metastases 10 years after radical surgery for the primary lesion in the neck. The lesions showed a good response to cyclophosphamide, vincristine, and dacarbazine chemotherapy. A beneficial therapeutic outcome by chemotherapy is extremely rare in patients with metastatic carotid body paraganglioma.

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HCV genotype 1 subtypes (1a and 1b): similarities and differences in clinical features and therapeutic outcome

Hepatology International ◽

10.1007/s12072-014-9556-2 ◽

2014 ◽

Vol 9 (1) ◽

pp. 52-57 ◽

Cited By ~ 5

Author(s):

A. Andriulli ◽

F. Morisco ◽

A. M. Ippolito ◽

V. Di Marco ◽

M. R. Valvano ◽

...

Keyword(s):

Clinical Features ◽

Therapeutic Outcome ◽

Genotype 1 ◽

Hcv Genotype ◽

Similarities And Differences ◽

Hcv Genotype 1 ◽

Hcv Genotype 1 Subtypes

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Gram Negative Bacillary Brain Abscess: Clinical Features And Therapeutic Outcome

The Internet Journal of Neurosurgery ◽

10.5580/2863 ◽

2007 ◽

Vol 4 (2) ◽

Keyword(s):

Brain Abscess ◽

Clinical Features ◽

Therapeutic Outcome ◽

Gram Negative

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HCV genotype 1a and 1b: Similarities and differences in clinical features, therapeutic outcome and predictors of response

Digestive and Liver Disease ◽

10.1016/j.dld.2014.01.100 ◽

2014 ◽

Vol 46 ◽

pp. e44

Author(s):

F. Morisco ◽

V. Di Marco ◽

N. Caporaso ◽

R. Granata ◽

S. Camera ◽

...

Keyword(s):

Clinical Features ◽

Therapeutic Outcome ◽

Hcv Genotype ◽

Genotype 1A ◽

Predictors Of Response ◽

Similarities And Differences

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Clinical features of essential thrombocythemia and primary myelofibrosis, depending on the molecular characteristics of disease

Terapevticheskii arkhiv ◽

10.17116/terarkh20178974-9 ◽

2017 ◽

Vol 89 (7) ◽

pp. 4-9

Author(s):

A L Melikyan ◽

I N Subortseva ◽

A B Sudarikov ◽

A M Kovrigina ◽

E A Gilyazitdinova ◽

...

Keyword(s):

High Risk ◽

Clinical Features ◽

Essential Thrombocythemia ◽

Triple Negative ◽

Gene Mutations ◽

Jak2 V617f ◽

Primary Myelofibrosis ◽

Molecular Characteristics ◽

Entire Group ◽

Thrombosis Risk

The aim of the present paper was to evaluate the clinical features and risk of thrombotic events (TE) in patients with essential thrombocythemia (ET) and primary myelofibrosis (PMF), depending on the molecular characteristics of disease. Clinical data and laboratory parameters were analyzed in 50 ET patients and 50 PMF ones who had been followed up at the Department for Standardization of Treatments, National Research Center for Hematology, Ministry of Health of the Russian Federation, from February 2015 to September 2016. The patients with ET and those with PMF were found to have a high risk of TE. The risk for TE in the patients with ET is higher (24% in the entire group) than in those with PMF (14% in the study group). In ET, there is a high thrombosis risk in the detection of JAK2 and CALR gene mutations as compared with triple-negative cases. The PMF patients with JAK2 V617F mutations are at high risk for TE compared to those who are CALR mutation carriers and in triple-negative cases. There was no significant association of TE with high thrombocytosis. A factor, such as age, was found to be of no negative prognostic value in the patients with PMF.

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Survival in Essential Thrombocythemia and Prefibrotic Primary Myelofibrosis - Correlation of Clinical Phenotype with Histomorphological Diagnosis

Blood ◽

10.1182/blood.v128.22.1932.1932 ◽

2016 ◽

Vol 128 (22) ◽

pp. 1932-1932

Author(s):

Georg Jeryczynski ◽

Albert Woelfler ◽

Bettina Gisslinger ◽

Martin Schalling ◽

Sonja Burgstaller ◽

...

Keyword(s):

Bone Marrow ◽

Overall Survival ◽

Clinical Features ◽

Correct Diagnosis ◽

Molecular Genetic ◽

Myeloproliferative Neoplasm ◽

Primary Myelofibrosis ◽

Diagnostic Systems ◽

Bone Marrow Histology ◽

Survival Patterns

Abstract Introduction The correct diagnosis of myeloproliferative neoplasm (MPN), especially the correct classification of early or prodromal stages, remains a matter of debate. While the 2008 WHOcriteriarecognize the existence of an early, prefibrotic primary myelofibrosis, other diagnostic systems make no such distinction between ET and prePMF. According to WHO criteria the accurate diagnosis is based mainly onhistomorphologicassessment of bone marrow (BM) biopsy samples. However, there is an ongoing debate if ET, prePMF, PV and overt PMF are, dependent from their molecular genetic phenotype, a continuum or rather independent entities. Methods From a multicenter database, we selected 251 patients with ET and 185 patients with prePMF, in which the diagnosis had been established by BM biopsy and in which clinical data at time of diagnosis and for follow up were available. We compared overall survival in patients from both entities in which common clinical features of prePMF were present at time of bone marrow biopsy. These were anemia (male <13 g/dL, female <12 g/dL),leukocyte counts ³ 11 G/L, elevated lactate dehydrogenase levels (LDH) as well as splenomegaly. Results Survival was substantially impaired in WHO-defined prePMF patients when compared to the ET cohort (Fig. 1). Median overall survival was significantly worse in patients with leukocytosis (9.3 vs. 14.9 years, 95% CI 6.9-11.6 vs. 9.1-20.7, p<0.001), elevated LDH levels (10.6 vs. 21.7 years, 95% CI 7.7-13.4 vs. 11.5-31.9, p<0.001) and splenomegaly (6.8 years vs. median not reached, 95% CI 6.8-10.8 vs.n.a.,p<0.001). Significance was narrowly missed in anemic patients (7.2 vs. 13.1 years, 95% CI 4.7-9.8 vs. 9.2-16.9, p=0.089). Discussion Our data illustrate, that the distinction of prePMF and ET patients based onhistomorphologicalcriteria by the WHO 2008 diagnostic criteria translates into different overall survival patterns in patients that may share some similar clinical features at diagnosis. This suggests that the underlying diseases are two different entities, with different underlying biology, that can both present with the same adverse clinical parameters. Further, these two entities can only be accurately differentiated by bone marrow histology. Therefore the concept that ET, WHO-defined prePMF and overt PMF form a continuum of the same disease, with prePMF just being an advanced stage of ET, may need revision in face of this study. Figure 1 Figure 1. Disclosures Burgstaller: Novartis: Consultancy, Honoraria. Geissler:Novartis: Honoraria. Gisslinger:AOP Orphan: Consultancy, Honoraria; Novartis: Consultancy, Honoraria; Baxalta: Consultancy, Honoraria.

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Incidence of Early Thrombosis in Myeloproliferative Neoplasms (MPN): A Prospective Analysis from the Gruppo Laziale of Ph-Negative MPN

Blood ◽

10.1182/blood.v128.22.1951.1951 ◽

2016 ◽

Vol 128 (22) ◽

pp. 1951-1951 ◽

Cited By ~ 1

Author(s):

Ambra Di Veroli ◽

Marianna De Muro ◽

Alessandro Andriani ◽

Malgorzata Trawinska ◽

Elena Rossi ◽

...

Keyword(s):

Clinical Features ◽

Myeloproliferative Neoplasms ◽

Thrombotic Event ◽

Major Complication ◽

Primary Myelofibrosis ◽

Lower Limbs ◽

High Morbidity ◽

Thrombotic Risk ◽

Reported Data ◽

Prospective Database

Abstract Background Thrombotic episodes are the major complication in the follow-up of Philadelphia negative Myeloproliferative Neoplasms (MPN), with high morbidity and mortality, as reported in several retrospective studies. At present, however, few prospective data are available on the early incidence of these complications. Methods To address this issue, we report on 1087 patients [M/F 508/579, median age 67.6 years, interquartile range (IQR) 55.2 - 75.9] with newly diagnosed MPN enrolled in the prospective database of our regional cooperative group since January 2011. Of them, 571 (52.5%) had Essential Thrombocythemia (ET), 303 (27.9%) Polycythemia Vera (PV) and 213 (19.6%) Primary Myelofibrosis (PMF). The main clinical features at diagnosis of the whole cohort and according to the different MPNs are reported in the Table 1. Results On the whole, 22 episodes of thrombotic complications were reported in 1087 patients (2.0%) at a median interval from diagnosis of 18.2 months (IQR 7.4 - 29.7): in particular, 15 (68.1%) were arterial (8 cerebral, 2 coronaric, 4 in the lower limbs, 1 splancnic) and 7 (31.9%) venous (5 in the lower limbs and 2 in the upper limbs). As to the incidence of early thrombosis in the different MPNs, they were 13/571 (2.2%) in ET patients, 5/303 (1.6%) in PV patients and 4/213 (1.8%) in PMF patients (p=0.810): median time from diagnosis to thrombotic event was also similar in the 3 MPNs (p=0.311). The 4-year cumulative Thrombosis-Free Survival (TFS) of the whole cohort was 97.3% (95%CI 96.0 - 98.6): there was no difference among the 3 MPNs as to 4-year TFS [96.7% (95%CI 94.8 - 98.6) in ET, 97.8% (95%CI 95.9 - 99.7) in PV and 98.7% (95%CI 96.9 - 100) in PMF, respectively, p=0.668). Several clinical features at diagnosis (age, gender, Hb levels, WBC and PLT counts, spleen enlargement, JAK-2 V617F mutation and previous thrombotic events) were evaluated for a role in predicting thrombotic events: only age (p=0.009) and previous thrombotic events (p=0.009) were significant. Conclusions The incidence of early thrombosis seems low in the first 4 years after diagnosis of MPN based on our prospective database, without any difference among ET, PV and PMF: it is worth of note that only age and previous thrombotic events had a predictive role, thus confirming many retrospective reported data and reinforcing the prognostic value of old scoring system for thrombotic risk in MPN. Table 1 Table 1. Disclosures Breccia: Ariad: Honoraria; Pfizer: Honoraria; Novartis: Consultancy, Honoraria; Bristol Myers Squibb: Honoraria; Celgene: Honoraria. Latagliata:Novartis: Consultancy, Honoraria; Bristol Myers Squibb: Honoraria; Celgene: Honoraria; Janssen: Consultancy, Honoraria; Shire: Honoraria.

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