RESUMO
BACKGROUND: Pneumocystis jirovecii pneumonia (PJP) is an opportunistic infection that primarily affects immunocompromised individuals. Typical symptoms of PJP include the subacute onset of dyspnea, nonproductive cough, and low-grade fever. In hematology patients, particularly those who are allogeneic stem cell transplant recipients, the disease often presents with a more aggressive clinical course. While hypercalcemia has been documented as a manifestation of PJP in some solid organ transplant recipients, it has not been reported in hematology or stem cell transplant patients. CASE PRESENTATION: Here, we present a case of PJP in a 56-year-old male allogeneic stem cell transplant recipient, who developed hypercalcemia and renal failure during the late post-transplant period. The patient had a history of allogeneic stem cell transplantation due to acute myeloid leukemia. He presented with symptoms of fatigue and weakness. Laboratory tests revealed hypercalcemia (13.8 mg/dL) and elevated serum creatinine levels (2.3 mg/dL). The patient was hospitalized, and despite initial treatment with hydration and furosemide, the hypercalcemia persisted, leading to the administration of denosumab. During follow-up, hypoxia was detected, and a chest CT scan revealed mosaic attenuation and ground-glass opacities. Bronchoscopy was performed, and PCR testing confirmed the presence of Pneumocystis jirovecii. Other causes of hypercalcemia were ruled out, with PTH measured at 13.8 pg/mL (normal range 15-65 pg/mL), albumin at 3.71 g/dL, 1.25-dihydroxy vitamin D3 at 96 ng/dL (normal range 26-95 ng/dL), and 25-hydroxy vitamin D at 32.5 ng/mL (normal range 20-40 ng/mL). A PET-CT scan demonstrated no pathological FDG uptake, with the exception of findings suggestive of a pulmonary infection. Following treatment with trimethoprim-sulfamethoxazole and denosumab, the patient's hypercalcemia and infection resolved. CONCLUSIONS: Although rare, PJP can present with hypercalcemia and kidney injury in allogeneic stem cell transplant recipients. Early diagnosis and treatment can improve both PJP and hypercalcemia.
Assuntos
Hipercalcemia , Pneumonia por Pneumocystis , Humanos , Hipercalcemia/etiologia , Masculino , Pessoa de Meia-Idade , Pneumonia por Pneumocystis/diagnóstico , Pneumonia por Pneumocystis/tratamento farmacológico , Pneumonia por Pneumocystis/etiologia , Pneumocystis carinii/isolamento & purificação , Transplante de Células-Tronco/efeitos adversos , Transplante Homólogo/efeitos adversos , Hospedeiro ImunocomprometidoRESUMO
INTRODUCTION: Imatinib is generally well tolerated by patients. The most common ophthalmic side effects are eyelid edema and periorbital edema. Other side effects which occur at rates of <1% include blepharitis, blurred vision, conjunctival hemorrhage, conjunctivitis, retinal hemorrhage, etc. An uncommon case is here reported of a 51-year-old male with chronic myeloid leukemia who developed vitreous hemorrhage due to imatinib after 9 months of treatment. CASE REPORT: A 51-year-old male with leukocytosis detected in the blood test examination was referred to the Hematology Department. The bone marrow biopsy result was compatible with chronic myeloid leukemia. Imatinib treatment (400â mg/day) was started. In the ninth month of imatinib treatment, the patient complained of a sudden decrease in vision. Vitreous hemorrhage was detected in the left eye and the patient underwent surgery. Vitreous hemorrhage recurred 1 month after the operation. On the fourth day after the discontinuation of imatinib treatment, the patient's ophthalmic complaints improved significantly. The Naranjo algorithm was applied and a score of 9 was detected. The vitreous hemorrhage of the patient was attributed to imatinib, and so the treatment of the patient was switched to bosutinib. DISCUSSION: Imatinib is an oral signal inhibitor that targets tyrosine kinase for BCR/ABL, platelet-derived growth factor, stem cell factor, and c-kit (CD117). The conjunctiva and sclera have a large amount of c-kit positive mast cells which are inhibited by imatinib. The inhibition of c-kit positive mast cells by imatinib may be responsible for further exposure of the conjunctival mucosa to injuries.
Assuntos
Antineoplásicos , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Leucemia Mielogênica Crônica BCR-ABL Positiva , Antineoplásicos/efeitos adversos , Benzamidas/uso terapêutico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/tratamento farmacológico , Edema/induzido quimicamente , Humanos , Mesilato de Imatinib/efeitos adversos , Leucemia Mielogênica Crônica BCR-ABL Positiva/diagnóstico , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Piperazinas , Pirimidinas/efeitos adversos , Hemorragia Vítrea/induzido quimicamente , Hemorragia Vítrea/tratamento farmacológicoRESUMO
INTRODUCTION: Synchronous detection of multiple myeloma and acute myeloid leukemia in a single patient is a rare coincidence. Treatment of these patients is still unclear, mostly based on acute myeloid leukemia strategies combined with bortezomib. CASE REPORT: A 72-year-old male with no medical history was investigated for pancytopenia. On medical examination, he was complicated with a wide and severe skin infection on arm. On examination of bone marrow aspirate, 25% myeloblasts infiltration and additional 10% plasma cells were seen. Acute myeloid leukemia was diagnosed and plasma cell proliferation was attributed to reactive plasmacytosis due to skin infection. However, flowcytometric studies and immunohistochemical examination revealed two different cell populations with 30-40% atypical plasma cells and >20% myeloblasts. Serum M-protein detected by serum electrophoresis test and immunofixation test revealed a monoclonal IgG lambda band. He was diagnosed with concurrent acute myeloid leukemia and multiple myeloma without history of chemotherapy.Management and outcome: The patient was initially treated with bortezomib and dexamethasone for the myeloma. Subsequently, azacitidine was administered subcutaneously for the acute myeloid leukemia treatment. The tru-cut biopsy of the lesion on his arm revealed suppurative inflammatory findings and no malign cells detected. Antibiotherapy was started according to susceptibility. He expired after three months of survival. DISCUSSION: The synchronous occurrence of these two different clonal hematological malignancies is rare in hematology practice. Patient-based prospective studies and case series are needed to guide diagnosis and treatment strategies. Furthermore, this report highlights the importance of ruling out reactive plasmacytosis in patients with hematological malignancy who developed severe infections.
Assuntos
Leucemia Mieloide Aguda/diagnóstico , Mieloma Múltiplo/diagnóstico , Idoso , Antibióticos Antineoplásicos/uso terapêutico , Antineoplásicos/uso terapêutico , Antineoplásicos Hormonais/uso terapêutico , Braço/patologia , Azacitidina/uso terapêutico , Biópsia , Medula Óssea/patologia , Bortezomib/uso terapêutico , Dexametasona/uso terapêutico , Evolução Fatal , Células Precursoras de Granulócitos/patologia , Humanos , Leucemia Mieloide Aguda/complicações , Leucemia Mieloide Aguda/tratamento farmacológico , Masculino , Mieloma Múltiplo/complicações , Mieloma Múltiplo/tratamento farmacológico , Proteínas do Mieloma , NecroseRESUMO
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. Herein, we aimed to report a diffuse large B-cell lymphoma (DLBCL) case that was presented as HLH. CASE REPORT: A 32-year-old man presented to a hospital with complaints of vomiting, nausea and diarrhea in October 2019. Fever and hepatosplenomegaly was detected in physical investigation. Bone marrow aspiration investigation revealed the hemaphagocytosis. HLH-2004 protocol was started for hemophagocytosis. Whole body magnetic resonance imaging (MR) revealed no lymphadenopathy. Bone marrow biopsy revealed high-grade B-cell lymphoma, favoring DLBCL. There were no pathologic cells in lumber puncture investigation. MANAGEMENT AND OUTCOME: He was diagnosed with secondary hemaphagocytic syndrome due to DLBCL, and chemotherapy was switched to rituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin (R-EPOCH) regimen. After three cycles of R-EPOCH chemotherapy regimen, complete remission was confirmed with positron emission tomography-computerised tomography (PET-CT) scan. DISCUSSION: Our patients' findings are suitable for six out of eight criteria of hemaphagocytic syndrome. The H-score of our patient was more than 250, reflecting the >99% probability of HLH syndrome. Compatible with literature knowledge, our patient had responded very well to etoposide-containing regimens. In our patient, no lymphadenopathy was detected by physical examination or MR scan, and the diagnosis of DLBCL was only made by the result of bone marrow investigation. In conclusion, herein, we have reported a DLBCL case that had presented with HLH, and clinicians should be aware that B-cell lymphomas may be the underlying cause of HLH.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfoma Difuso de Grandes Células B/complicações , Adulto , Biópsia , Exame de Medula Óssea , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Febre/etiologia , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Imageamento por Ressonância Magnética , Masculino , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Prednisona/administração & dosagem , Rituximab/administração & dosagem , Vincristina/administração & dosagem , Imagem Corporal TotalRESUMO
INTRODUCTION: All-trans retinoic acid (ATRA) is a physiological metabolite of vitamin A and it is used for the treatment of acute promyelocytic leukemia (APL). Hypercalcemia is a rare side effect of ATRA and it may be potentiated after interaction of ATRA with azole group antifungals. Herein, we have reported an APL case with hypercalcemia that is caused by the interaction of ATRA and posaconazole. CASE REPORT: A 49-year-old female patient was diagnosed as APL after the examinations performed upon the detection of pancytopenia when she had presented with the complaints of widespread bruising and fever. After the initiation of posaconazole and ATRA, her serum calcium levels begin to increase (10.3 to 11.1mg/dl). Her vitamin D level was 21.9 ng/ml and PTH 17.8 pg/ml, both were in the normal ranges. The Drug Interaction Probability Scale score of our case was calculated as 6, indicating that the probable adverse drug reaction. Therefore, the high level of serum calcium was attributed to the interaction between ATRA and posaconazole. MANAGEMENT & OUTCOME: Although hypercalcemia with ATRA and other antifungal agents have been previously reported in the literature, this is the first report of hypercalcemia with the concomitant use of ATRA and posaconazole. DISCUSSION: This case highlights the importance of monitoring ATRA's side effects when it is used in combination with drugs inhibiting the cytochrome P450 enzymes. In conclusion, the concomitant use of posaconazole and ATRA may lead to hypercalcemia and serum calcium levels return to normal ranges with the discontinuation of these drugs.
Assuntos
Hipercalcemia , Leucemia Promielocítica Aguda , Feminino , Humanos , Hipercalcemia/induzido quimicamente , Leucemia Promielocítica Aguda/tratamento farmacológico , Pessoa de Meia-Idade , Tretinoína/efeitos adversos , Triazóis/efeitos adversosRESUMO
Hemophagocytic Lymphohistiocytosis (HLH) is a reactive disorder of the mononuclear phagocytic system characterized by increased histiocytic proliferation, activation and hemaphagocytosis. The underlying etiology may be genetic (primary) or acquired (secondary). Secondary causes include drugs, autoimmune diseases, malignancies and infections of which EBV is the most common. A 28-year old male patient who was a shepherd with no known concomitant comorbid disease was admitted to the Emergency Department with the complaints of abdominal pain, fever, severe fatigue. Physical examination revealed high fever, hepatosplenomegaly and laboratory examination revealed pancytopenia, hyperferritinemia and hypertriglyceridemia. Hemophagocytes were observed in the bone marrow biopsy and the patient was diagnosed as HLH. The patient was treated with cyclosporine A, dexamethasone, intravenous immunoglobulin (IvIg) and etoposide according to the HLH 2004 protocol. Coxiella burnetii was detected in the serological evaluation of the etiology and doxycycline was added to the current treatment. Fever was controlled in the second week of the treatment and the patient was discharged after complete recovery of the cytopenia in the fourth week. In the outpatient setting, treatment was completed in 8 weeks and follow-up of the patient is still ongoing without medication. To the best of our knowledge, this is the first case from Turkey of HLH secondary to Q-fever which was treated and managed successfully. Since the mortality of HLH is quite high, the etiology should be determined as soon as possible to be able to provide appropriate treatment.
Assuntos
Linfo-Histiocitose Hemofagocítica/etiologia , Febre Q/complicações , Adulto , Humanos , Linfo-Histiocitose Hemofagocítica/patologia , Masculino , Doenças RarasRESUMO
INTRODUCTION: Dasatinib is a potent tyrosine-kinase inhibitor which is used for chronic myeloid leukemia treatment. Pleural effusion is a frequent side effect in patients during dasatinib treatment. Pulmonary arterial hypertension is a rare and life-threatening adverse event of dasatinib. The relationship between dasatinib and autoimmune disorders is unclear, but there are reports of possible mechanisms that have triggered autoimmunity by dasatinib. CASE REPORT: A 53-year-old male was diagnosed with chronic myeloid leukemia and initiated imatinib mesylate as a treatment. Imatinib was changed to dasatinib as the patient was unresponsive in the first year of treatment. In the fourth year of dasatinib when chronic myeloid leukemia was in both hematological and cytogenetical remission, the patient presented with bilateral massive exudative pleural effusion. Echocardiography was consistent with pericardial effusion with right ventricle enlargement and normal left-side cardiac function. Pulmonary arterial hypertension was diagnosed with high systolic pulmonary arterial pressure. When he had fever and arthralgia, further investigation showed positivity of anti-nuclear antibodies (1/160 titer) and anti-RNP/Sm, which have high specificity for the diagnosis of Systemic Lupus Erythematosus (SLE). MANAGEMENT AND OUTCOME: Dasatinib was discontinued and nilotinib was initiated. As the pleural effusion persisted despite diuretics and methylprednisolone, mycophenolate mofetil was initiated as a steroid-sparing immune-suppressive agent. The lupus-like symptoms disappeared, and antibodies became undetectable after dasatinib discontinuation. Pericardial effusion improved and pleural effusion did not relapse. DISCUSSION: Screening for auto-antibodies may be recommended for patients with a history or symptoms of autoimmune disease before starting dasatinib. All patients who develop pleural effusion while on dasatinib treatment should be investigated for antibodies for lupus.
Assuntos
Dasatinibe/efeitos adversos , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Lúpus Eritematoso Sistêmico/induzido quimicamente , Dasatinibe/administração & dosagem , Ecocardiografia , Humanos , Mesilato de Imatinib/uso terapêutico , Masculino , Pessoa de Meia-Idade , Derrame Pericárdico/induzido quimicamente , Derrame Pleural/induzido quimicamente , Inibidores de Proteínas Quinases/uso terapêutico , Pirimidinas/administração & dosagemRESUMO
BACKGROUND: Patients with cancer are at increased risk of thromboembolic complications. There is no evidence-based guideline on the use of routine prophylaxis in hematological malignancies except in patients with multiple myeloma. The purpose of this study was to determine the incidence and risk factors of thrombosis and suggest a rationale for primary thromboprophylaxis in acute leukemia and lymphoma patients. PATIENTS AND METHODS: A retrospective study was conducted on newly-diagnosed acute leukemia and lymphoma patients who presented at our institution from November 2009 to March 2018. The study included a total of 157 patients with acute leukemia and 238 patients with lymphoma. The groups were analyzed to determine the incidence and risk factors of thromboembolic complications. RESULTS: The incidence of all thrombotic complications was 10.12% (40/395) including 11.4% (18/157) in patients with acute leukemia and 9.2% (22/238) in patients with lymphoma. The majority of events occurred in the first 6 months. Acute leukemia patients with thrombosis had a higher number of comorbidities than those without thrombosis (p < 0.05). Lymphoma patients with thrombotic complications had significantly higher beta-2-microglobulin and lactate dehydrogenase levels compared to those without thrombosis (p < 0.05). Major bleeding events developed in five (3.1%) acute leukemia patients and two (0.8%) lymphoma patients. All the major bleeding events occurred when the patients were thrombocytopenic (platelet < 50,000/mm3). CONCLUSIONS: Acute leukemia patients with any comorbidity and lymphoma patients with higher lactate dehydrogenase and beta-2-microglobulin are at high risk of developing thromboembolic complications. The prophylactic use of anticoagulant should be considered for those patients especially in the first 6 months.
Assuntos
Anticoagulantes/uso terapêutico , Leucemia/tratamento farmacológico , Linfoma/tratamento farmacológico , Trombose/epidemiologia , Adulto , Idoso , Feminino , Hemorragia/induzido quimicamente , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de RiscoRESUMO
Diffuse large B cell lymphoma (DLBCL) constitutes the most frequent subtype of all non-Hodgkin's lymphomas. DLBCL is an aggressive disease and extranodal involvement is seen in approximately 30% of patients and most common extranodal sites are gastointestinal tract and skin. Skin involvement may be either primary or secondary. Secondary cutaneous lymphoma has a worse prognosis. The case is here reported of a 56-year old male DLBCL patient with cutaneous lesions and aggressive clinical course. The patient had no skin lesions at diagnosis and during follow up and treatment period, skin, cerebrospinal fluid and bone marrow involvement was occurred. Salvage chemotherapy and autologous stem cell transplantation was planned but the patient died before the second cycle of salvage chemotherapy. In contrast to primary cutaneous lymphoma, which tends to be more indolent, secondary skin involvement is associated with unfavourable prognosis. In conclusion it should be kept in mind that skin can be involved in lymphoma patients and in these cases, skin biopsy should be performed rapidly.
Assuntos
Progressão da Doença , Linfoma Difuso de Grandes Células B/complicações , Tratamento Farmacológico/métodos , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de DoençaRESUMO
The case is here presented of a 70-year old male patient with rare coexistence of Kaposi Sarcoma and resistant Thrombotic Thrombocytopenic Purpura (TTP). The Kaposi lesions were determined before the diagnosis of TTP and were exacerbated after receiving TTP-associated immunosuppressive therapy, in particular associated with rituximab. TTP in this case was resistant to conventional therapies such as steroid and plasma exchange and current immunosuppressive (rituximab, cyclophosphamide, vincristin) treatments. Novel treatment agents consisting of bortezomib and eculizumab given to the patient were also ineffective. To the best of our knowledge, this case presents the first case of coexistence of TTP and Kaposi sarcoma from Turkey and the challenge of refractory TTP management.
Assuntos
Púrpura Trombocitopênica Trombótica/etiologia , Púrpura Trombocitopênica Trombótica/terapia , Sarcoma de Kaposi/complicações , Idoso , Humanos , Masculino , Púrpura Trombocitopênica Trombótica/patologia , Sarcoma de Kaposi/patologiaRESUMO
Transverse myelitis is a quite rare complication of hematopoietic stem cell transplantation. The case is here reported of a 49 year old male with diffuse large B cell lymphoma in complete remission who developed transverse myelitis after autologous stem cell transplantation. The patient presented with numbness and sensory loss of the bilateral lower extremities and difficulty in urinating on the 20th day after cell transplantation. Millimetric hyperintensity was detected in the C5-C6 and T2-T5 segments of the spinal cord on cervical and thoracic vertebral magnetic resonance imaging. Treatment was initiated of pulse steroid and intravenous immunoglubulin followed by plasmapheresis and cyclophosphamide due to inadequate response. The patient then started a rehabilitation program and was discharged in the 9th month after stem cell transplantation when most of the symptoms were relieved. To the best of our knowledge, this is the first case reported in literature of TM development after autologous stem cell transplantation.
Assuntos
Ciclofosfamida/administração & dosagem , Transplante de Células-Tronco Hematopoéticas , Linfoma Difuso de Grandes Células B , Imageamento por Ressonância Magnética , Mielite Transversa , Plasmaferese , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/terapia , Masculino , Pessoa de Meia-Idade , Mielite Transversa/diagnóstico por imagem , Mielite Transversa/etiologia , Mielite Transversa/terapia , Transplante AutólogoRESUMO
BACKGROUND: Immunophenotyping has a central role in CLL. However, CLL is a very heterogenous disease, both morphologically and immunophenotypically; thus, its diagnosis may prove a challenge. We investigated CD81 ex-pression in the differential diagnosis of CLL and MCL. METHODS: We retrospectively examined CD81 expression with 8 color Multiparameter Flow cytometry devices in 101 CLL and 19 MCL cases. RESULTS: We found negative CD81 expression in CLL cases whereas it was positive in MCL cases. CONCLUSIONS: Our results suggest that CD81 may be a valuable marker for the differential diagnosis of CLL. We are of the opinion that it should be definitely included in the diagnostic algorithm for CLL.
Assuntos
Leucemia Linfocítica Crônica de Células B/diagnóstico , Tetraspanina 28/metabolismo , Diagnóstico Diferencial , Humanos , Leucemia Linfocítica Crônica de Células B/metabolismo , Estudos RetrospectivosRESUMO
BACKGROUND: Prophylaxis is strongly recommended in patients with hematological malignancy who are usually at higher risk for infection and neutropenic fever. It is still unclear whether or not there is a definite need for antimicrobial prophylaxis in intermediate-risk hematology patients such as those with lymphoma. METHODS: A retrospective analysis was made of patients admitted from January 2009 to December 2017 to the Hematology Department of Diskapi Yildirim Beyazit Training and Research Hospital, a tertiary referral hospital in Ankara, Turkey. The study included patients who were diagnosed with any type of lymphoma and given chemotherapy. Routine antimicrobial prophylaxis was administered to 127 lymphoma patients, and not to 65 lymphoma patients. These two groups were compared in respect of the incidence of total infection episodes (IE), febrile neutropenia episodes, and nonneutropenic clinically documented infection episodes. RESULTS: For all patients with lymphoma and subtypes of non-Hodgkin lymphoma or Hodgkin lymphoma, no significant difference was determined between the groups in respect of the total incidence of IE, febrile neutropenia and nonneutropenic clinically documented infection both during the first-line chemotherapy and throughout the total follow-up period (p > 0.05). Patients with prophylaxis had a higher incidence of IE, which was treated with parenteral antibiotics both during the first-line chemotherapy and throughout the total follow-up period (p < 0.05). CONCLUSION: Antimicrobial prophylaxis was seen to have no effect on the total incidence of infection episode and febrile neutropenia. Therefore, the routine use of antimicrobial prophylaxis should not be recommended for patients with lymphoma.
Assuntos
Anti-Infecciosos/uso terapêutico , Antibioticoprofilaxia , Infecções Bacterianas/prevenção & controle , Neutropenia Febril/prevenção & controle , Linfoma/tratamento farmacológico , Adolescente , Adulto , Idoso , Antineoplásicos/efeitos adversos , Infecções Bacterianas/induzido quimicamente , Neutropenia Febril/induzido quimicamente , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Adulto JovemRESUMO
Sarcoidosis is known to be associated with higher incidence of solid tumors and hematological malignancies. ALK(-) CD30(+) anaplastic large cell lymphoma is a type of non-Hodgkin lymphoma showing poor prognosis, and seldom co-occurs with sarcoidosis. As this rare and highly mortal disease did not respond to classical chemotherapies and showed remission with brentuxumab vedontin treatment, we are presenting our first case reported from Turkey hoping to contribute to the literature.
Assuntos
Imunoconjugados/administração & dosagem , Linfoma Anaplásico de Células Grandes/tratamento farmacológico , Sarcoidose/tratamento farmacológico , Brentuximab Vedotin , Humanos , Antígeno Ki-1/metabolismo , Masculino , Turquia , Adulto JovemRESUMO
Hemophilia is a hereditary disease with impaired blood coagulation due to a genetic deficiency of blood coagulation factors. The development of inhibitors further complicates the course of the disease and management. The case is here reported of a haemophilia patient who presented with coexisting development of high titer inhibitor with Gastrointestinal Stromal Tumor (GIST) diagnosis and was admitted with upper gastrointestinal system bleeding. The patient had no prior history of inhibitor presence. During all procedures including surgery, excellent hemostasis was achieved with rFVIIa treatment and no hemorrhagic complication was observed. To the best of our knowledge, this constitutes the first reported case of GIST associated with inhibitor development in a hemophilia A patient.
Assuntos
Tumores do Estroma Gastrointestinal/etiologia , Hemofilia A/complicações , Adulto , Tumores do Estroma Gastrointestinal/patologia , Hemofilia A/patologia , Humanos , MasculinoRESUMO
Various side effects associated with dimethyl sulfoxide (DMSO) which is used for cryopreservation of bone marrow or peripheral blood progenitor cells (PBPCs) have been reported. Among the central nervous system side effects the epileptic seizures, stroke, transient and temporary leucoencephalopathy, and global amnesia are well known. Herein we report a 52-year-old man who experienced tonic-clonic seizure within minutes after the initiation of DMSO cryopreserved autologous PBPC infusion. Unfortunately, he also developed cardiac arrest and required intubation for ventilation after the seizure. Pathophysiology of acute neurological and cardiac toxicity is unclear, but may also be idiosyncratic. Clinicians should be aware of the toxicity of cryoprotectant agents during PBSC infusion. Determining the risk factors associated with increased DMSO toxicity and taking preventive actions is utmost important.
Assuntos
Parada Cardíaca/induzido quimicamente , Parada Cardíaca/complicações , Transplante de Células-Tronco de Sangue Periférico/efeitos adversos , Células-Tronco de Sangue Periférico/citologia , Convulsões/induzido quimicamente , Convulsões/complicações , Dimetil Sulfóxido , Evolução Fatal , Humanos , Masculino , Pessoa de Meia-Idade , Transplante Autólogo/efeitos adversosRESUMO
BACKGROUND: Hairy cell leukemia (HCL) is a rare mature B-cell malignancy that is primarily treated with purine analogues. However, relapse remains a significant challenge, prompting the search for alternative therapies. The BRAF V600E mutation prevalent in HCL patients provides a target for treatment with vemurafenib. PATIENTS AND METHODS: This multicenter retrospective study included nine patients with relapsed/refractory (R/R) HCL from six different centers. Patient data included demographics, prior treatments, clinical outcomes, and adverse events. RESULTS: Patients received different treatment regimens between centers, including vemurafenib alone or in combination with rituximab. Despite the differences in protocols, all patients achieved at least a partial response, with seven patients achieving a complete response. Adverse events were generally mild with manageable side effects. The absence of myelotoxic effects and manageable side effects make BRAF inhibitors attractive, especially for patients ineligible for purine analogues or those with severe neutropenia. CONCLUSION: Single agent vemurafenib or in combination with rituximab appears to be a promising therapeutic option for R/R HCL. Further research is needed to establish standardized treatment protocols and to investigate long-term outcomes.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica , Leucemia de Células Pilosas , Rituximab , Vemurafenib , Humanos , Leucemia de Células Pilosas/tratamento farmacológico , Leucemia de Células Pilosas/patologia , Vemurafenib/administração & dosagem , Vemurafenib/uso terapêutico , Vemurafenib/efeitos adversos , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Rituximab/efeitos adversos , Masculino , Pessoa de Meia-Idade , Feminino , Estudos Retrospectivos , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Adulto , Proteínas Proto-Oncogênicas B-raf/genética , Proteínas Proto-Oncogênicas B-raf/antagonistas & inibidores , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/patologia , Resultado do Tratamento , Idoso de 80 Anos ou mais , Resistencia a Medicamentos AntineoplásicosRESUMO
Central nervous system (CNS) involvement in multiple myeloma (MM) is a rare and challenging complication associated with poor prognosis and limited treatment options. Emerging T-cell directing therapies, such as bispecific antibodies (bsAbs) and chimeric antigen receptor T cells (CAR-T), have shown remarkable success in treating MM, but their efficacy in CNS involvement remains unclear. Elranatamab, a humanized bispecific antibody targeting B-cell maturation antigen (BCMA) and CD3-expressing T cells, has demonstrated promising results in relapsed refractory MM. However, its efficacy in treating CNS-MM has not been reported. We present a case of a 37-year-old male MM patient with CNS involvement who has been successfully treated with Elranatamab.
Assuntos
Anticorpos Biespecíficos , Mieloma Múltiplo , Masculino , Humanos , Adulto , Mieloma Múltiplo/tratamento farmacológico , Imunoterapia Adotiva/métodos , Anticorpos Biespecíficos/uso terapêutico , Antígeno de Maturação de Linfócitos BRESUMO
OBJECTIVE: Acute myeloid leukemia (AML) is a hematological malignancy that frequently affects elderly population. With introducing the hypomethylating agents (HMAs) in elderly AML treatment, survival rates and quality of life have improved. However, long-term management in elderly and frail patients is still a challenge. In the present study, we aimed to determine whether HMA maintenance therapy is required until disease progression in frail and elderly AML patients by examining with a real-life data. METHODS: In a multicenter study, we analyzed non-promyelocytic elderly AML patients who were treated with first-line azacitidine or decitabine monotherapy in two different groups, retrospectively. While patients were treated with HMA until progression in the maintenance group, 6+3 cycles of azacitidine or decitabine were administered as a standard care of elderly AML patients in the non-maintenance group. Survival outcomes were compared between the groups. RESULTS: HMA therapy was maintained until progression in 20 patients, and HMA therapy was terminated after 6+3 cycles in 21 patients. Patients received a median of 6 (1-14) HMA cycles during follow-up time. The median 7.5 months of overall survival were observed (2-17 months) in maintenance and 3 months (1-13 months) in non-maintenance groups (p=0.001). CONCLUSION: Despite long-term exposure to HMA may appear as a risk factor for complications and toxicities in elderly and frail AML patients, the maintenance of therapy until disease progression provides a significant survival advantage. Therefore, we suggest that HMA therapy should continue until disease progression regardless the sort of HMA.
RESUMO
Lymphomatoid granulomatosis (LG) is Epstein-Barr virus associated and aggressive B cell lymphoproliferative disease. The most common sites of involvement are lungs, skin, kidneys, liver and central nervous system. The clinical presentation of pulmonary LG may mimic infectious diseases, malignancies or vasculitis. While treatment approach of low grade disease is watch and wait, patients with advanced stage require aggressive treatment with chemotherapy. Patients with hematological malignancy as well as solid tumors are at increased risk of venous thromboembolic events (VTE). We reported here in a case of pulmonary LG who was complicated with VTE during treatment with chemo-immunotherapy After 4 cycles of R-CHOP, she achieved complete remission for LG and was followed up without relapse for 2 years. She was anticoagulated with Low-Molecular-Weight Heparin (LMWH) during chemotherapy period, and the thrombus improved over the next several weeks. While on this paper written, patient completed her pregnancy successfully under anticoagulation prophylaxis.