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1.
Ann Hematol ; 102(12): 3587-3591, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37783854

RESUMEN

Since 2006, combined graft-versus-host disease (GVHD) prophylaxis with ATG Grafalon has been our department's base of peri-transplant supportive care. This recent retrospective study included 398 patients who underwent their first allogeneic hematopoietic stem cell transplantation after receiving a defined dose of ATG Grafalon. Our observations recorded reduced incidence of severe acute and chronic GVHD without negative impact on overall survival in a nonselected group with standard and uniform GVHD prophylaxis.


Asunto(s)
Síndrome de Bronquiolitis Obliterante , Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Humanos , Estudios Retrospectivos , Incidencia , Enfermedad Injerto contra Huésped/epidemiología , Enfermedad Injerto contra Huésped/etiología , Enfermedad Injerto contra Huésped/prevención & control , Trasplante Homólogo/efectos adversos , Suero Antilinfocítico/uso terapéutico , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Acondicionamiento Pretrasplante/efectos adversos
2.
Vnitr Lek ; 69(E-3): 4-15, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37468330

RESUMEN

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications, which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. Common symptoms at onset include lower back, abdominal or flank pain, and constitutional symptoms such as malaise, fever, and anorexia and weight loss. Pain is frequently referred to the hip, to the groin and to the lateral regions of the leg, with nocturnal exacerbations, and typically does not modify with position. We report a case of 56 year-old male with recurrent lower back pain and lower abdominal pain. Contrast-enhanced computed tomography and was suggestive of retroperitoneal fibrosis and unilateral ureteral occlusion. Histologic examination with immunohistochemical staining for IgG4 demonstrate IgG4-related retroperitoneal fibrosis. Therapy was started with prednison 1 mg/kg, but the tolerance of this dose was poor. Therefore the therapy was switched to combination of rituximab 375 mg/ m2 on day 1, cyclophosphamide 300 mg/m2 mg infusion and dexamethasone 20 mg total dose infusion on day 1 and 15 in 28 days cycle. FDG-PET/CT control in fourth month showed residual accumulation of FDG in retroperitoneal fibrotic mass, and therefore the therapy was prolonged to 8 month. The subjective symptoms of this diseases disappeared in the 8th month. Then the maintenance therapy, administration of rituximab in 6 month interval, was started. The activity of this disease be further evaluated by FDG-PET/CT imagination. Glucocorticoids are considered the cornerstone of therapy. The use of other immunosuppressive agents, including cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil and biological agents such as rituximab, tocilizumab and infliximab and sirolimus have been reported as a valuable option mostly in case reports, cases series and small studies. This agents allowed to reduce cumulative dose of glucocorticoids and its adverse effects. Therefore in our patients we preferred combination of rituximab cyclophosphamide s dexamethasone with lover dose of prednisonem. This combination is preferable for patients who cannot tolerate glucocorticoids or who are likely to suffer from significant glucocorticoids -related toxicity.


Asunto(s)
Enfermedad Relacionada con Inmunoglobulina G4 , Fibrosis Retroperitoneal , Masculino , Humanos , Persona de Mediana Edad , Glucocorticoides/uso terapéutico , Rituximab/uso terapéutico , Fibrosis Retroperitoneal/complicaciones , Fibrosis Retroperitoneal/tratamiento farmacológico , Fibrosis Retroperitoneal/diagnóstico , Tomografía Computarizada por Tomografía de Emisión de Positrones , Enfermedad Relacionada con Inmunoglobulina G4/complicaciones , Enfermedad Relacionada con Inmunoglobulina G4/tratamiento farmacológico , Fluorodesoxiglucosa F18/uso terapéutico , Ciclofosfamida/uso terapéutico , Dexametasona/uso terapéutico , Inmunoglobulina G/uso terapéutico
3.
Vnitr Lek ; 68(E-5): 4-19, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36283812

RESUMEN

Immunoglobulin G4- related disease (IgG4-RD) is a rare systemic fibro-inflammatory disorder. Autoimmune pancreatitis is the most frequent manifestation of IgG4-RD. However, IgG4-RD can affect any organ such as salivary glands, orbits, retroperitoneum, prostate and many others. Recent research enabled a clear clinical and histopathological description of IgG4-RD and in 2019 four Clinical phenotypes of IgG4-related disease were described. Diagnosis is based on morphological examination with typical findings of lymphoplasmocellular inflammation, storiform fibrosis and obliterative phlebitis in IgG4-RD biopsies and the tissue invading plasma cells largely produce IgG4. Elevated serum IgG4 levels are found in many but not all patients. New diagnostic criteria for IgG4-RD have been published recently in 2019 and 2021. This review summarizes current knowledge on pathophysiology, clinical manifestations, diagnosis and differential diagnosis of IgG4-RD from the point of view 2022 and in next article brings overview of the IgG4-RD therapy.


Asunto(s)
Enfermedades Autoinmunes , Enfermedad Relacionada con Inmunoglobulina G4 , Masculino , Humanos , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/patología , Diagnóstico Diferencial , Inmunoglobulina G , Inflamación , Fibrosis , Enfermedades Raras/diagnóstico , Enfermedades Autoinmunes/diagnóstico
4.
Vnitr Lek ; 68(E-2): 11-21, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36208940

RESUMEN

Langerhans cell histiocytosis (LCH) is a rare condition with incidence in adults 1-2/1 million, wherein Langerhans cells proliferate abnormally, adversely impacting organs including most frequently bones, skin, lungs, pituitary gland, lymph nodes, gums and other organs. The LCH course varies widely among patients from a self-limiting condition, to one that progresses. But LCH only very rarely culminates in death. To aim of this text is to review all possible symptoms and manifestations of this disease.


Asunto(s)
Histiocitosis de Células de Langerhans , Adulto , Histiocitosis de Células de Langerhans/diagnóstico , Histiocitosis de Células de Langerhans/metabolismo , Histiocitosis de Células de Langerhans/terapia , Humanos , Ganglios Linfáticos/patología , Enfermedades Raras
5.
Vnitr Lek ; 68(E-6): 15-22, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36316207

RESUMEN

Immunoglobulin IgG4 related disease (IgG4-RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the beginning of this century only. Evolving therapy is reviewed in this paper. Glucocorticoids are first choice drug but long administration of glucocorticoids is connected with many adverse effects. In case of combination glucocorticoids and immunosuppressive agents lower doses of glucocorticoids are needed, the response rate is higher and therapy is better tolerated. Rituximab is drug, that is possible use as monotherapy or in combination with glucocorticoids and immunosuppressive drugs. Only one study compared two immunosuporessive drugs, mycophenolate mofetil and cyclophosphamide. The response rated was similar but remissions were longer after glucocorticoids with cyclophosphamide then glucocorticoids with mycofenolat mofetil. No other comparative study of combination of various imunossupressive drugs with glucocorticoids was published. Rituximab has high number (90 %) of response rate in monotherapy, but can be used in combination with glucocorticoids and immunosuppressives. Rituximab is now preferred and recommended for maintenance therapy administered in 6-month interval. In case of advanced disease, we prefer therefore combination of rituximab, cyclofosphamide and dexamethasone for initial therapy followed by maintenance with rituximab in 6 months interval. There are two new drugs under investigation abatacept and dupilimab with promising results. Although we have very intensive therapies for good results of therapy early diagnosis before irreversible fibrotic changes in IgG4-RD involved organs is still needed.


Asunto(s)
Enfermedad Relacionada con Inmunoglobulina G4 , Humanos , Enfermedad Relacionada con Inmunoglobulina G4/tratamiento farmacológico , Rituximab/uso terapéutico , Inmunoglobulina G , Resultado del Tratamiento , Inmunosupresores/uso terapéutico , Glucocorticoides/uso terapéutico , Ciclofosfamida
6.
Klin Onkol ; 35(4): 315-322, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35989089

RESUMEN

BACKGROUND: Lenalidomid ranks among immunomodulatory drugs. There are a few of the more common side effects, like a higher risk of venous trombembolism or diarrhea. Other side effects are rare. The hyperbilirubinemia described in this article can be assigned to them. In our case, the increase of bilirubin was associated with unrecognized Gilbert syndrome. CASE DESCRIPTION: We report a patient with multiple myeloma and necrobio-tic xanthogranuloma (NXG) of the skin and liver. After the treatment with bortezomib, lenalidomid and dexamethasone, complete remission was attained after 4 cycles with decrease of monoclonal immunoglobulin to an unmeasurable concentration. At the same time, the dis-appearance of cutaneous and hepatic lesions of NXG on FDG-PET/CT was evident. The administration of bortezomib was stopped after 8 cycles and only continued with lenalidomide as a maintenance therapy. However, after four cycles of this therapy, bilirubin increased above the upper limit and the increase continued till the 11th month of lenadomide administration, when bilirubin reached the highest concentration of 75 μmol/l (more than the three-fold of the upper limit, grade III toxicity). The patient had asymptomatic hyperbilirubinemia with no underlying liver disease or renal impairment while being on lenalidomide therapy. Genetic studies proved mutation; insertion in the promotor gene UGT1A1 typical for Gilbert syndrome. Hyperbilirubinemia may be attributed to the unmasking of previously undia-gnosed Gilbert syndrome. Therefore, the therapy with lenalidomide was interrupted after 11 months. The bilirubin level decreased after the discontinuation of the drug. CONCLUSION: NXG disappeared after fulfilling complete remission of multiple myeloma with disappearance of monoclonal immunoglobulin. This observation supports the hypothesis that monoclonal immunoglobulin has a crucial role in the ethiopathogenesis of NXG and suggests the treatment of monoclonal gammopathy if present in a patient with NXG, hoping that this will result in xantogranuloma disappearance.


Asunto(s)
Enfermedad de Gilbert , Mieloma Múltiple , Xantogranuloma Necrobiótico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Bilirrubina , Bortezomib/uso terapéutico , Dexametasona/uso terapéutico , Enfermedad de Gilbert/tratamiento farmacológico , Humanos , Hiperbilirrubinemia/tratamiento farmacológico , Lenalidomida/uso terapéutico , Mieloma Múltiple/tratamiento farmacológico , Mieloma Múltiple/patología , Xantogranuloma Necrobiótico/diagnóstico , Xantogranuloma Necrobiótico/tratamiento farmacológico , Tomografía Computarizada por Tomografía de Emisión de Positrones
7.
Neoplasma ; 69(5): 1008-1018, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35900317

RESUMEN

Chimeric antigen receptor (CAR)-T cells are a new treatment modality in various hematological malignancies, including relapsed/refractory multiple myeloma (RRMM). RRMM patients have a poor prognosis, and their treatment options are limited. Currently available data from clinical trials on CAR-T cell therapy have demonstrated efficacy and manageable toxicity in RRMM. The CAR-T cells in RRMM mostly focus on already known cellular targets, such as B-cell maturation antigen (BCMA). CAR-T cells focusing on other targets have been analyzed in various clinical trials as well. Cytokine release syndrome (CRS), specific neurotoxicity, and hematological toxicity are the main adverse events (AE); according to the clinical trials, they are mostly mild with a low incidence of grade 3 or higher toxicities. The autologous CAR-T cell therapy against BCMA (ide-cel and cilta-cel) shows the best efficacy with an overall response rate and a median progression-free survival in RRMM. Both ide-cel and cilta-cel have already been approved by the FDA. Currently, the main controversies in the routine use of CAR-T cells are high treatment costs and unknown long-term efficacy. In this review, we summarize the current overview of CAR-T cell therapies in RRMM in 2021 with various targets for CAR-T cells and their efficacy, safety, and possible limitations. Future prospective clinical trials are needed to clarify the optimal role of CAR-T cells in MM therapy.


Asunto(s)
Mieloma Múltiple , Receptores Quiméricos de Antígenos , Antígeno de Maduración de Linfocitos B/uso terapéutico , Humanos , Mieloma Múltiple/tratamiento farmacológico , Receptores Quiméricos de Antígenos/uso terapéutico , Linfocitos T/patología
8.
Vnitr Lek ; 68(1): 41-53, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35459346

RESUMEN

Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric (UCD) or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8-negative/idiopathic (iMCD). Treatment of iMCD is challenging, and outcomes can be poor. In this paper, we briefly report about symptoms of iMCD and about the International, evidencebased consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease and International evidence based consensus treatment guidelines for idiopathic multicentric Castleman disease.


Asunto(s)
Enfermedad de Castleman , Herpesvirus Humano 8 , Enfermedad de Castleman/diagnóstico , Enfermedad de Castleman/patología , Enfermedad de Castleman/terapia , Consenso , Humanos
9.
Ann Hematol ; 100(10): 2541-2546, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34309714

RESUMEN

Infections are the primary cause of morbidity and mortality in multiple myeloma (MM) patients (pts). The aim of our retrospective analysis was to evaluate incidence and course of COVID-19 infection in a cohort of 351 MM outpatients treated with novel drugs. COVID-19 disease was detected in 50/351 pts (14%); median age was 68 years. Gender, ISS stage, and last treatment lines were as follows: male 32, female 18; ISS-I 19, ISS-II 20, ISS-III 11; daratumumab-based 15, lenalidomide-based 12, bortezomib-based 17, other 6. Positive PCR test at COVID-19 diagnosis was present in all pts; anti-myeloma treatment was interrupted. Hospitalizations for COVID-19 pneumonia were necessary for 28/50 pts (56%), 18/50 pts (36%) in standard unit (SU) 10/50 pts (20%) in intensive care unit (ICU), and 9/50 pts (18%) died. The statistically significant parameters for COVID-19 hospitalization were as follows: responsive versus non-responsive disease (p = 0.027), ECOG performance status 0-2 versus ≥ 3 (p = 0.014), presence of comorbidities (0-1 versus ≥ 2, p = 0.043). The statistically significant factors for COVID-19 death were as follows: ECOG 0-2 versus ≥ 3 (p = 0.001), presence of comorbidities (0-1 versus ≥ 2, p = 0.007), serious course of COVID-19 disease with ICU hospitalization (SU versus ICU, p = 0.001). None of the other studied risk factors was associated with poor outcome (age, gender, ISS stage, immunoparesis, type of anti-myeloma treatment). Full recovery from COVID-19 infection was observed in 41/50 pts (82%) in median of 32 days. The course of COVID-19 disease in MM pts was mostly moderate or serious with 56% of hospitalizations and 18% of deaths.


Asunto(s)
Anticuerpos Monoclonales/uso terapéutico , Antineoplásicos/uso terapéutico , Bortezomib/uso terapéutico , COVID-19/etiología , Lenalidomida/uso terapéutico , Mieloma Múltiple/complicaciones , Mieloma Múltiple/tratamiento farmacológico , Adulto , Anciano , Anciano de 80 o más Años , COVID-19/complicaciones , COVID-19/diagnóstico , Femenino , Hospitalización , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , SARS-CoV-2/aislamiento & purificación , Resultado del Tratamiento
10.
Vnitr Lek ; 67(E-4): 9-12, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34275313

RESUMEN

Lymphangiomatosis is rare disease, we can find this entity in differential diagnosis of osteolytic leasions of bones of unknown origin. Typical sign for lymphangiomatosis is proliferation of lymphatic tissue with production of lymphangiomas in various organs and systems. Clinical manifestation of disease is variable, involvement of lungs and bone is typical. In our article we present recent classification of lymphatic tissue neoplasias, their clinical symptoms and treatment possibilities.


Asunto(s)
Linfangioma , Vasos Linfáticos , Huesos , Diagnóstico Diferencial , Humanos , Linfangioma/diagnóstico por imagen , Vasos Linfáticos/diagnóstico por imagen , Enfermedades Raras
11.
Vnitr Lek ; 67(E-3): 15-23, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34171947

RESUMEN

Transformation of IgM-MGUS into Waldenström´s macroglobulinemia in two of six patients treated for Schnitzler´s syndrome Schnitzler´s syndrome is a very rare, adult-onset, apparently acquired autoinflammatory disease. Chronic urticarial rash and symptoms of systemic inflammation including fever, arthralgia and bone pain with the presence of monoclonal immunoglobulin M (IgM), rarely IgG, are among hallmarks of the disease. We performed a retrospective study of 6 patients (5 men, 1 woman) diagnosed with Schnitzler´s syndrome fulfilling the Strasbourg criteria who had been treated at our centre in the University Hospital Brno from 2007 to 2021. Median age at diagnosis was 54 (45-67) years, median follow up was 8 (3-14) years. All 6 patients had IgM κ monoclonal gammopathy, increased CRP and/or erythrocyte sedimentation rate and arthralgia or bone pain, 4 patients suffered from fever, three had leucocytosis 10 × 109/L and lymphadenopathy was found in one patient. 18FDG-PET/CT scan with low-dose total body CT became a part of the initial baseline assessment in 5 patients with suspected Schnitzler´s syndrome, while Na18F-PET/CT was used in one patient to confirm the presence of osteosclerotic leasions as a criterion of the disease. All patients had osteosclerotic or hyperostotic bone lesions detected by low-dose CT examination, with increased 18FDG uptake in illiac and femoral bone marrow. The patient with Na18F-PET/CT scan revealed intensive abnormal tracer uptake with Na18F-PET/CT being more sensitive for detection of osteosclerotic lesions in Schnitzler´s syndrome than 18FDG-PET/CT. All patients were treated with daily subcutaneous anakinra without any adverse events, with excellent clinical results. We observed complete disappearance of urticaria and other symptoms persisting during years of anakinra administration. IgM-MGUS transformed into Waldenström´s macroglobulinemia in two of six patients, but only one patient developed symptoms requiring RBD (Rituximab, Bendamustin, and Dexamethasone) treatment, which induced almost complete remission of the disease. Successful RBD therapy enabled to prolong intervals of maintenance anakinra from 24 to 48 hours with almost complete control of urticarial rash and other symptoms. We suggest close monitoring of patients with Schnitzler´s syndrome to early capture potential transformation into Waldenström´s macroglobulinemia with succesful treatment of both conditions.


Asunto(s)
Síndrome de Schnitzler , Macroglobulinemia de Waldenström , Adulto , Femenino , Humanos , Inmunoglobulina M , Masculino , Tomografía Computarizada por Tomografía de Emisión de Positrones , Estudios Retrospectivos
12.
Vnitr Lek ; 67(3): 157-164, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34171955

RESUMEN

Three adult patients with confirmed Erdheim-Chester disease (ECD) are followed at our department. Cladribine in monotherapy or in combination with cyclophosphamide were used for first line therapy. The median number of cycles of cladribine or cladribine and cyclophosphamide was 7 (range 6-8). In two cases complete response was achieved, in one case this therapy achieved no response. The duration of response is in one case 11 years, in second case the follow up is too short for evaluation of response duration. In case of no-response to cladribine and cyclophosphamide stabilisation of disease was achieved with anakinra. The tolerance was good without any toxicity grade II and higher. Cladribin and cyclophosphamide is one option for treatment of Erdheim-Chester disease.


Asunto(s)
Cladribina , Enfermedad de Erdheim-Chester , Adulto , Ciclofosfamida , Humanos , Proteína Antagonista del Receptor de Interleucina 1 , Inducción de Remisión
13.
Neoplasma ; 68(3): 519-527, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-33618517

RESUMEN

Despite the high efficacy of current induction regimens, most multiple myeloma (MM) patients relapse over time. The link between changes in the immune system and the prognosis of the disease is still not entirely clear. Therefore, we analyzed whether the pattern of bone marrow (BM) lymphocytes during routine BM examination after autologous stem cell transplant (ASCT) is related to disease prognosis or MRD negative complete remission. From 2009 to 2018, 98 MM patients underwent routine BM testing after the first ASCT. Using multi-parametric flow cytometry, twelve BM lymphocyte subtypes were analyzed. In 60% of patients who achieved a complete response (CR), MRD by flow cytometric analysis (sensitivity threshold 10-6) was evaluated. We found an association of relative proportion of BM lymphocyte subtypes with treatment response, progression-free survival (PFS), overall survival (OS), and minimal residual disease (MRD) negativity. Higher relative proportion of memory B cells was associated with inferior median PFS [HR 1.089 (95% CI: 1.023-1.160), p=0.008] and median OS [HR 1.170 (95% CI: 1.074-1.274), p<0.001]. In non-responding patients (minimal response and worse), higher proportion of memory B cells was found when compared to patients achieving CR [3.8% (range 0.5-35.0) vs. 1.0% (range 0.1-12.5); p=0.001]. No significant association of BM lymphocyte subtypes proportion with MRD negative CR was found. Our results show that changes in BM lymphocyte subsets including memory B cells may have prognostic value in MM patients after ASCT.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Mieloma Múltiple , Autoinjertos , Humanos , Linfocitos , Mieloma Múltiple/terapia , Recurrencia Local de Neoplasia , Neoplasia Residual , Pronóstico , Inducción de Remisión , Trasplante Autólogo , Resultado del Tratamiento
14.
Vnitr Lek ; 67(7): 419-424, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-35459360

RESUMEN

Hereditary hemorrhagic telangiectasia also known as Osler-Weber-Rendu syndrome, is an disorder that causes abnormal blood vessel formation with bleeding. Inhibition of angiogenesis amelioretes bleeding complication. Anti-angiogenic agents such as bevacizumab, aflibercept, thalidomid, lenadomid and other new anti-angiogenic thyrosinkinase inhibitors, as well as sirolimus and takrolimus have emerged as a promising systemic or local therapy in reducing bleeding complications but are not curative. Other pharmacological agents include iron supplementation, antifibrinolytics and hormonal treatment. This review concentrates on new anti-agioproliferative drugs with effect in HHT- discusses the new biology of HHT, management issues that face the practising hematologist, and considerations of future directions in HHT treatment.


Asunto(s)
Telangiectasia Hemorrágica Hereditaria , Inhibidores de la Angiogénesis/uso terapéutico , Bevacizumab/uso terapéutico , Hemorragia/complicaciones , Humanos , Síndrome , Telangiectasia Hemorrágica Hereditaria/complicaciones , Telangiectasia Hemorrágica Hereditaria/tratamiento farmacológico
15.
Vnitr Lek ; 67(8): 465-473, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-35459366

RESUMEN

Castleman disease (CD) includes a group of rare and heterogeneous disorders with characteristic lymph node histopathological abnormalities. CD can occur in a single lymph node station, which is referred to as unicentric CD (UCD). CD can also involve multicentric lymphadenopathy and inflammatory symptoms - multicentric Castleman disease. The first-ever diagnostic and treatment guidelines were recently developed for UCD and published 2020. Complete surgical resection is often curative and is therefore the preferred first-line therapy, if possible. The management of unresectable UCD is more challenging. Existing evidence supports that asymptomatic unresectable UCD may be observed. The anti-interleukin-6 monoclonal antibody siltuximab should be considered for unresectable UCD patients with an inflammatory syndrome. Unresectable UCD that is symptomatic because of compression of vital neighbouring structures may be rendered amenable to resection by medical therapy (rituximab, steroids), radiotherapy, or embolization. In this article, we report about the symptoms of this disease and about the diagnostics recommendation published in the International, evidence-based consensus diagnostic criteria for HHV-8-negative/ idiopathic multicentric Castleman disease and about the therapeutic recommendation published in International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease published in the year 2020.


Asunto(s)
Antineoplásicos , Enfermedad de Castleman , Antineoplásicos/uso terapéutico , Enfermedad de Castleman/tratamiento farmacológico , Enfermedad de Castleman/terapia , Consenso , Humanos , Rituximab/uso terapéutico
16.
Vnitr Lek ; 67(6): 339-344, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-35459376

RESUMEN

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is an autosomal dominant disorder that causes abnormal blood vessel formation. Patients with HHT may have telangiectasias and later may develop arteriovenous malformations in various organs. Pacients suffer from many complications caused by the malformations and therefore by patients with HHT must by performed screening of this arteriovenous malformations. Optimal treatment of this malformations is best delivered throught a multidisciplinary approach. Farmacological treatment is described in next paper.


Asunto(s)
Malformaciones Arteriovenosas , Telangiectasia Hemorrágica Hereditaria , Malformaciones Arteriovenosas/complicaciones , Malformaciones Arteriovenosas/diagnóstico , Malformaciones Arteriovenosas/genética , Humanos , Telangiectasia Hemorrágica Hereditaria/complicaciones , Telangiectasia Hemorrágica Hereditaria/diagnóstico , Telangiectasia Hemorrágica Hereditaria/genética
17.
Vnitr Lek ; 67(6): 352-356, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-35459378

RESUMEN

Necrobiotic xanthogranuloma (NXG) is a rare chronic condition, belonging to the group non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly monoclonal gammopathy, MGUS and multiple myeloma. The case reported here NXG was diagnosed after 1 years of evolution in patient with asymptomatic multiple myeloma. After treatment with bortezomib, lenalidomid and dexamethasone, there was evident abrupt decrease of monoclonal immunoglobulin to not measurable level (complete remission of multiple myeloma) and in the same time was evident disappearance of cutaneous and hepatic lesions of NXG on FDG-PET/CT. The etiopathogenetic association of monoclonal immunoglobulin with NXG is documented in this case report with disappearance of NXG in the time of disappearance of monoclonal immunoglobulin.


Asunto(s)
Mieloma Múltiple , Xantogranuloma Necrobiótico , Bortezomib/uso terapéutico , Dexametasona/uso terapéutico , Humanos , Inmunoglobulinas , Mieloma Múltiple/complicaciones , Mieloma Múltiple/tratamiento farmacológico , Xantogranuloma Necrobiótico/complicaciones , Xantogranuloma Necrobiótico/diagnóstico , Xantogranuloma Necrobiótico/tratamiento farmacológico , Tomografía Computarizada por Tomografía de Emisión de Positrones
18.
Vnitr Lek ; 66(6): 19-27, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33380149

RESUMEN

The histiocytoses are rare disorders characterized by the accumulation of cells thought to be derived from dendritic cells or macrophages. Their clinical behaviour ranges from mild to disseminated and, sometimes, life-threatening forms. The incidence of this diseases is much smaller, then the incidence of diseases derived from lymphocytic or myeloid lineage. Langerhans cell histiocytosis is most frequent disease from this group. The last version of WHO classification from 2017 and last version of classification published by Histiocyte Society is summarised in this paper.


Asunto(s)
Histiocitosis de Células de Langerhans , Neoplasias , Linaje de la Célula , Células Dendríticas , Histiocitos , Humanos , Macrófagos , Organización Mundial de la Salud
20.
Bone Marrow Transplant ; 55(2): 356-366, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-31534192

RESUMEN

Plerixafor plus granulocyte-colony stimulating factor (G-CSF) enhances the mobilization of hematopoietic stem cells (HSCs) for collection and subsequent autologous hematopoietic stem cell transplantation (HSCT) in patients with multiple myeloma (MM). This international, multicenter, noninterventional registry study (NCT01362972), evaluated long-term outcomes for MM patients who received plerixafor versus other mobilization regimens. The comparisons were: G-CSF + plerixafor (G-CSF + P) versus G-CSF-; G-CSF + P versus G-CSF + chemotherapy (G-CSF + C); and G-CSF + P + C versus G-CSF + C. Propensity score matching was used to balance groups. Primary outcome measures were progression free survival (PFS), overall survival (OS), and cumulative incidence of relapse (CIR) after transplantation. After propensity matching, 77 versus 41 patients in the G-CSF + P versus G-CSF cohorts, 129 versus 129 in the G-CSF + P versus G-CSF + C cohorts, and 117 versus 117 in the G-CSF + P + C versus G-CSF + C cohorts were matched, respectively. Propensity score matching resulted in a smaller sample size and imbalances were not completely overcome. For both PFS and OS, the upper limits of the hazard ratio 95% confidence intervals exceeded prespecified boundaries; noninferiority was not demonstrated. CIR rates were higher in the plerixafor cohorts. G-CSF + P remains an option for the mobilization of HSCs in poor mobilizers with MM with no substantial differences in PFS, OS, and CIR in comparison with other regimens.


Asunto(s)
Compuestos Heterocíclicos , Mieloma Múltiple , Bencilaminas , Ciclamas , Movilización de Célula Madre Hematopoyética , Humanos , Mieloma Múltiple/terapia , Recurrencia Local de Neoplasia , Sistema de Registros
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