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1.
Ceska Gynekol ; 89(3): 215-218, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38969516

RESUMO

Abruptio placenta can be a catastrophic event with a high association with adverse maternal and fetal outcomes. We present a case of massive abruptio placenta occurring in a young asymptomatic mother at 30 weeks' gestation. Although electronic fetal monitoring and ultrasound allowed a prompt diagnosis of an 8 × 5 cm retroplacental hematoma, the fetus died at the time of emergency cesarean section. The fetus was intubated, but could not be resuscitated. Histologic examination of the placenta documented thinning and stacked hypercapillarized villi, with syncytial buds and foci of fibrinoid necrosis in the presence of hyaline streaks on both the maternal and fetal sides.


Assuntos
Descolamento Prematuro da Placenta , Humanos , Feminino , Gravidez , Descolamento Prematuro da Placenta/diagnóstico , Adulto , Cesárea , Terceiro Trimestre da Gravidez , Morte Fetal , Evolução Fatal
2.
BMJ Case Rep ; 17(7)2024 Jul 03.
Artigo em Inglês | MEDLINE | ID: mdl-38960429

RESUMO

Mass-forming phenotypes of IgG4-related disease (IgG4-RD) mimic malignancy and histological confirmation can be challenging. A woman in her 70s with HIV infection presented with painless obstructive jaundice and weight loss. Magnetic resonance imaging was suggestive of unresectable cholangiocarcinoma. Tumour markers and serum IgG4 were normal. Percutaneous liver biopsy was consistent with IgG4-RD inflammatory pseudotumour, with complete response to glucocorticoid therapy. Two years later, a new episode of obstructive jaundice occurred, with CT showing a solid lesion in the head of the pancreas with double duct sign and encasement of the portal vein. Re-induction therapy was tried without response. Fine-needle biopsy was consistent with pancreatic cancer. Supportive care was offered and the patient died 8 months later, with no signs of disease progression on subsequent imaging. We discuss the challenges of IgG4-RD diagnosis and treatment and the differential diagnosis between mass-forming phenotypes and malignancy, highlighting the difficulties in managing such patients.


Assuntos
Colangiocarcinoma , Doença Relacionada a Imunoglobulina G4 , Neoplasias Pancreáticas , Humanos , Feminino , Doença Relacionada a Imunoglobulina G4/diagnóstico , Diagnóstico Diferencial , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/patologia , Idoso , Colangiocarcinoma/diagnóstico , Evolução Fatal , Fenótipo , Imunoglobulina G/sangue , Imageamento por Ressonância Magnética , Icterícia Obstrutiva/etiologia , Tomografia Computadorizada por Raios X , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/patologia , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/diagnóstico por imagem
3.
Rinsho Ketsueki ; 65(6): 492-497, 2024.
Artigo em Japonês | MEDLINE | ID: mdl-38960646

RESUMO

A 69-year-old man presented with lumbago and was diagnosed with multiple myeloma (IgD-λ type, R-ISS stage II) with bone-destructive lesions in the lumbar spine and sacrum. Chromosome analysis showed t (8;14)(q24;q32) and t (11;14)(q13;q32). Treatment with daratumumab, lenalidomide, and dexamethasone resulted in partial response, but the disease relapsed, with a copy number increase in t (11;14) and abnormal amplification of the 1q21 region. The patient was treated for CMV enteritis, and was admitted to the hospital due to sudden abdominal pain. Gastrointestinal perforation was diagnosed by CT scan showing free air and wall thickening in the small intestine. Emergency surgery was performed, and the tumors in the perforated area were positive for CCND1 but negative for MYC on immunostaining. The patient's general condition did not improve after the surgery and he died. Pathological autopsy revealed extramedullary infiltration of multiple organs in addition to the small intestine. Extramedullary infiltration is thought to be caused by clonal evolution, and further research is warranted to clarify its pathogenesis and establish effective therapeutic strategies in high-risk patients.


Assuntos
Mieloma Múltiplo , Humanos , Masculino , Mieloma Múltiplo/patologia , Mieloma Múltiplo/diagnóstico , Idoso , Evolução Fatal , Translocação Genética , Cromossomos Humanos Par 14 , Cromossomos Humanos Par 11
4.
Hinyokika Kiyo ; 70(3): 71-75, 2024 Mar.
Artigo em Japonês | MEDLINE | ID: mdl-38961698

RESUMO

We report a case of small cell carcinoma of the urethra with inguinal lymph node metastases. A 50- year-old female patient presented with gross hematuria. Cystoscopy and computed tomography (CT) revealed a tumor surrounding the urethra and an inguinal lymphadenopathy. Biopsy of the urethral tumor demonstrated small cell carcinoma. Four courses of chemotherapy with etoposide and cisplatin, followed by 66 Gy of irradiation achieved complete remission. Unfortunately, 14 months later, positroemission-CT scan revealed recurrence of inguinal lymph node metastases. Although seven courses of chemotherapy with nogitecan were carried out, a new metastatic bone tumor developed. Amrubicin was administered as a third-line treatment, but was canceled after one course because of side effects. The patient died at 39 months after diagnosis. Small cell carcinoma of urethra with metastases has extremely poor prognosis, as is demonstrated by this case.


Assuntos
Carcinoma de Células Pequenas , Neoplasias Uretrais , Humanos , Feminino , Pessoa de Meia-Idade , Carcinoma de Células Pequenas/diagnóstico por imagem , Carcinoma de Células Pequenas/terapia , Carcinoma de Células Pequenas/patologia , Neoplasias Uretrais/patologia , Neoplasias Uretrais/diagnóstico por imagem , Neoplasias Uretrais/terapia , Metástase Linfática , Evolução Fatal , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Tomografia Computadorizada por Raios X
5.
Can Vet J ; 65(7): 661-666, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38952763

RESUMO

Carcinosarcomas are very rare tumors in dogs. Although carcinosarcomas with melanocytic differentiation arising from organs other than the thymus have been described in humans, this type of tumor has not been reported in dogs in any part of the body. We observed such a tumor in the cranial mediastinum of an 11-year-old spayed female dachshund. The dog was admitted to the clinic because of coughing, sporadic regurgitation, and dyspnea. Thoracic ultrasonography and computed tomography revealed a large mediastinal mass that was surgically removed via sternotomy. The tumor was of thymic origin and demonstrated 3 distinct components: an epithelial component positive for pancytokeratin (AE1/AE3) and high molecular weight cytokeratin (CK5/CK6) with some cystic spaces; a mesenchymal component positive for vimentin; and in association with the epithelial part, a minor melanocytic component positive for Melan A. Histologic metastasis of the epithelial and melanocytic components was present within a tracheobronchial lymph node. The dog died 105 d after surgery, after an episode of acute dyspnea. Key clinical message: To the authors' knowledge, this is the first report of thymic carcinosarcoma with melanocytic differentiation.


Carcinosarcome thymique avec différenciation mélanocytaire chez un chienLes carcinosarcomes sont des tumeurs très rares chez le chien. Bien que des carcinosarcomes avec différenciation mélanocytaire provenant d'organes autres que le thymus aient été décrits chez l'homme, ce type de tumeur n'a été rapporté chez le chien dans aucune partie du corps. Nous avons observé une telle tumeur dans le médiastin cránien d'une femelle teckel stérilisée de 11 ans. Le chien a été admis à la clinique en raison de toux, de régurgitations sporadiques et de dyspnée. L'échographie thoracique et la tomodensitométrie ont révélé une masse médiastinale importante qui a été retirée chirurgicalement par sternotomie. La tumeur était d'origine thymique et présentait 3 composantes distinctes : une composante épithéliale positive pour la pancytokératine (AE1/AE3) et la cytokératine de haut poids moléculaire (CK5/CK6) avec quelques espaces kystiques; un composant mésenchymateux positif à la vimentine; et en association avec la partie épithéliale, un composant mélanocytaire mineur positif pour Melan A. Des métastases histologiques des composants épithéliaux et mélanocytaires étaient présentes dans un ganglion lymphatique trachéobronchique. Le chien est décédé 105 jours après l'intervention chirurgicale, à la suite d'un épisode de dyspnée aiguë.Message clinique clé :À la connaissance des auteurs, il s'agit du premier cas de carcinosarcome thymique avec différenciation mélanocytaire.(Traduit par Dr Serge Messier).


Assuntos
Carcinossarcoma , Doenças do Cão , Neoplasias do Timo , Animais , Cães , Doenças do Cão/patologia , Doenças do Cão/cirurgia , Doenças do Cão/diagnóstico , Feminino , Carcinossarcoma/veterinária , Carcinossarcoma/patologia , Carcinossarcoma/cirurgia , Carcinossarcoma/diagnóstico , Neoplasias do Timo/veterinária , Neoplasias do Timo/cirurgia , Neoplasias do Timo/patologia , Neoplasias do Timo/diagnóstico , Evolução Fatal , Melanócitos/patologia
7.
Rev Esp Patol ; 57(3): 211-216, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38971621

RESUMO

This report details a rare case of squamous cell carcinoma (SCC) in the stomach, a condition accounting for only a fraction of gastric carcinomas. A 46-year-old male patient with dysphagia, abdominal pain, and haematemesis was diagnosed with primary gastric SCC displaying aggressive metastasis, an exceptionally low-incidence condition affecting mainly males in their sixth decade of life. Primary gastric SCC, though clinically similar to adenocarcinoma, involves a bleaker prognosis, lacking standardized treatment protocols. Histopathology and imaging confirmed the diagnosis, highlighting the challenges in managing advanced cases. Palliative chemotherapy showed partial remission but led to severe neuropathy. The case underscores the urgent need for research to understand the pathogenesis, effective management, and therapeutic targets for primary gastric SCC, emphasizing its scarcity and poor prognosis in medical literature. Increased clinical awareness and ongoing research are crucial for improving outcomes in such rare presentations.


Assuntos
Carcinoma de Células Escamosas , Neoplasias Gástricas , Humanos , Masculino , Neoplasias Gástricas/patologia , Pessoa de Meia-Idade , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/secundário , Evolução Fatal , Cuidados Paliativos
8.
Dermatol Online J ; 30(2)2024 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-38959923

RESUMO

Hailey-Hailey disease (HHD) is a rare, autosomal dominant genodermatosis caused by a mutation of the ATP2C1 gene and presenting as an erosive dermatosis, particularly in the intertriginous areas. Generalized HHD is a rare variant. We present a case of widespread, recalcitrant HHD in a middle-aged woman with a fatal outcome. No other underlying dermatosis was identified, with the possible exception of drug sensitivity to carbamazepine. Diagnosis of HHD was confirmed by histology and genetic studies which showed a c.2395C>T mutation in the ATP2C1 gene. Concurrent pemphigus was excluded. Cases of generalized HHD are extremely rare and present a challenge in diagnosis and management. Increased awareness of this severe clinical variant is needed to improve quality of care for patients with this form of HHD.


Assuntos
ATPases Transportadoras de Cálcio , Mutação , Pênfigo Familiar Benigno , Humanos , Pênfigo Familiar Benigno/genética , Pênfigo Familiar Benigno/patologia , Pênfigo Familiar Benigno/tratamento farmacológico , Feminino , ATPases Transportadoras de Cálcio/genética , Pessoa de Meia-Idade , Evolução Fatal
9.
J Med Case Rep ; 18(1): 301, 2024 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-38951939

RESUMO

BACKGROUND: Pulmonary aspergillosis is a prevalent opportunistic fungal infection that can lead to mortality in pediatric patients with underlying immunosuppression. Appropriate and timely treatment of pulmonary aspergillosis can play a crucial role in reducing mortality among children admitted with suspected infections. CASE PRESENTATION: The present study reports three cases of inappropriate treatment of pulmonary aspergillosis caused by Aspergillus flavus in two Iranian pediatric patients under investigation and one Afghan patient. Unfortunately, two of them died. The cases involved patients aged 9, 1.5, and 3 years. They had been diagnosed with pulmonary disorders, presenting nonspecific clinical signs and radiographic images suggestive of pneumonia. The identification of A. flavus was confirmed through DNA sequencing of the calmodulin (CaM) region. CONCLUSION: A. flavus was the most prevalent cause of pulmonary aspergillosis in pediatric patients. Early diagnosis and accurate antifungal treatment of pulmonary aspergillosis could be crucial in reducing the mortality rate and also have significant potential for preventing other complications among children. Moreover, antifungal prophylaxis seems to be essential for enhancing survival in these patients.


Assuntos
Antifúngicos , Aspergillus flavus , Aspergilose Pulmonar , Humanos , Aspergillus flavus/isolamento & purificação , Antifúngicos/uso terapêutico , Criança , Masculino , Pré-Escolar , Aspergilose Pulmonar/tratamento farmacológico , Aspergilose Pulmonar/diagnóstico , Lactente , Feminino , Evolução Fatal , Irã (Geográfico)
10.
J Assoc Physicians India ; 72(7): 97-99, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38990594

RESUMO

Podophyllin poisoning is a rare but serious emergency, often fatal, that involves multiple systems, predominantly the nervous system. Usually, it results from the resin extract derived from certain plants, and podophyllotoxin is the most toxic chemical present in it. The toxicity mechanisms involve suppression of cellular nucleoside transport and microtubule disruption. Due to the delayed onset of symptoms, there can be a delay in diagnosing and treating the condition. In addition, the initial and dominant central nervous system (CNS) symptoms can be mistaken for CNS infection or brainstem stroke. Therefore, early diagnosis (based on a high degree of suspicion, and circumstantial evidence) and prompt treatment (primarily decontamination and supportive care) are crucial to prevent unfavorable outcomes. We present a rare case of a 23-year-old male who accidentally ingested podophyllin and initially experienced severe neurological symptoms, subsequently developing into multisystem involvement and culminating in death within 4 days of its ingestion.


Assuntos
Podofilina , Humanos , Masculino , Adulto Jovem , Evolução Fatal , Podofilina/intoxicação
11.
Diagn Pathol ; 19(1): 96, 2024 Jul 09.
Artigo em Inglês | MEDLINE | ID: mdl-38982440

RESUMO

BACKGROUND: Mycobacterium avium complex (MAC) is an uncommon clinical pathogen, especially in the central nervous system (CNS), and carries a poor prognosis. MAC infections commonly present as immune reconstitution disease (IRD) in HIV patients. Herein, we report a case of intracranial infection caused by MAC in an AIDS patient without disseminated MAC (DMAC) and immune reconstitution inflammatory syndrome (IRIS). CASE PRESENTATION: A 31-year-old HIV-positive male presented us with progressively worsening CNS symptoms, and neuroimaging revealed ring-enhancing lesions. The intracranial lesions worsened after the empirical therapy for toxoplasma encephalitis and fungal infection. Due to the rapid progression of the disease, the patient died. Mycobacterium avium was the only pathogen in brain tissue after cultures and molecular biology tests. CONCLUSION: MAC infection in CNS is challenging to diagnose in HIV patients. Our findings emphasize that obtaining tissue samples and applying molecular biology methods is essential to help diagnose the patient as soon as possible to receive adequate treatment.


Assuntos
Infecções Oportunistas Relacionadas com a AIDS , Complexo Mycobacterium avium , Infecção por Mycobacterium avium-intracellulare , Humanos , Masculino , Adulto , Infecção por Mycobacterium avium-intracellulare/diagnóstico , Infecção por Mycobacterium avium-intracellulare/microbiologia , Infecção por Mycobacterium avium-intracellulare/complicações , Infecções Oportunistas Relacionadas com a AIDS/microbiologia , Infecções Oportunistas Relacionadas com a AIDS/diagnóstico , Evolução Fatal , Complexo Mycobacterium avium/isolamento & purificação , Síndrome da Imunodeficiência Adquirida/complicações , Encéfalo/patologia , Encéfalo/microbiologia
12.
Vet Med Sci ; 10(4): e1520, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38979745

RESUMO

An 11-year-old female cinnamon cockatiel (Nymphicus hollandicus) was presented with a coelomic distention. Dystocia was suspected, given its previous history of a calcium-deficient diet and multiple instances of nonobstructive dystocia. Exploratory coeliotomy revealed a large intraluminal mass extending through the magnum to the uterus (shell gland). Metastasis and multiorgan involvement were not seen. Histopathologically, malignant and invasive fascicles of spindle cells were associated with abundant myxoid matrix and hypocellular areas. Multinucleation, bizarre cells and atypical mitotic figures were prominent. Masson's trichrome staining verified the muscular origin, and the myxoid matrix was demonstrated utilizing Alcian blue. The neoplastic cells exhibited alpha-smooth muscle actin and desmin immunoreactivity and were negative for vimentin. Thus, the patient was diagnosed with oviductal and uterine myxoid leiomyosarcoma (LMS). The patient survived 34 days post-surgery before death associated with suspected enteritis. Myxoid LMS is an extremely rare neoplasm in animals. To our knowledge, myxoid LMS has not been reported previously in pet birds.


Assuntos
Doenças das Aves , Cacatuas , Leiomiossarcoma , Oviductos , Neoplasias Uterinas , Feminino , Animais , Leiomiossarcoma/veterinária , Leiomiossarcoma/patologia , Leiomiossarcoma/cirurgia , Neoplasias Uterinas/veterinária , Neoplasias Uterinas/patologia , Neoplasias Uterinas/cirurgia , Neoplasias Uterinas/diagnóstico , Doenças das Aves/patologia , Doenças das Aves/cirurgia , Doenças das Aves/diagnóstico , Oviductos/patologia , Evolução Fatal
13.
Rev Fac Cien Med Univ Nac Cordoba ; 81(2): 381-390, 2024 06 28.
Artigo em Espanhol | MEDLINE | ID: mdl-38941227

RESUMO

Introduction: Cerebral gas embolism is an unusual but extremely serious condition that occurs when air is introduced into the arterial or venous circulation of the brain. Although rare, it can lead to significant neurological deficits and even the death of the patient. Clinical Case: 76-year-old patient with pre-existing diffuse interstitial lung disease, who experienced a massive stroke due to spontaneous pneumomediastinum. Her presentation included confusion, seizures, and motor weakness. Imaging tests revealed air bubbles in the cerebral sulci and hypodense areas in the cerebellum and parietooccipitals. In addition, pneumothorax and air in the upper mediastinum were noted on chest radiographs and chest CT scan. Despite therapeutic measures such as hyperbaric oxygen, the patient unfortunately died due to multiple organ failure. Discussion: The diagnosis of cerebral gas embolism generally involves performing a cerebral computed tomography, which is highly sensitive for detecting the presence of air in the cerebral vessels. Management includes monitoring of vital and neurological signs, as well as specific measures such as airway closure, venous catheter aspiration, Trendelenburg positioning, and hyperbaric oxygen. Conclusion: Cerebral gas embolism is a potentially fatal condition that requires a brain computed tomography for diagnosis and it is vitally important to know the prevention measures to avoid the appearance of this complication and also to know the general measures to adopt when it occurs.


Introducción: La embolia gaseosa cerebral es una afección inusual pero extremadamente grave que se produce cuando se introduce aire en la circulación arterial o venosa del cerebro. Aunque poco común, puede derivar en déficits neurológicos significativos e incluso la muerte del paciente. Caso Clínico: Paciente de 76 años con una enfermedad pulmonar intersticial difusa preexistente, que experimentó un ictus masivo debido a un neumomediastino espontáneo. Su presentación incluyó confusión, convulsiones y debilidad motora. Las pruebas de imagen revelaron burbujas de aire en los surcos cerebrales y áreas hipodensas en el cerebelo y parietooccipitales. Además, se observó neumotórax y aire en el mediastino superior en las radiografías de tórax y la tomografía torácica. A pesar de las medidas terapéuticas como el oxígeno hiperbárico, la paciente lamentablemente falleció debido al fallo multiorgánico. Discusión: El diagnóstico de embolia gaseosa cerebral generalmente implica la realización de una tomografía computarizada cerebral, que es altamente sensible para detectar la presencia de aire en los vasos cerebrales. El manejo incluye el control de las constantes vitales y neurológicas, así como medidas específicas como cierre de la entrada de aire, aspiración de catéteres venosos, posicionamiento de Trendelenburg y oxígeno hiperbárico. Conclusión: La embolia gaseosa cerebral es una afección potencialmente mortal que requiere una tomografía computarizada cerebral para el diagnóstico y de vital importancia conocer las medidas de prevención para evitar la aparición de esta complicación y así mismo conocer las medidas generales a adoptar cuando ésta se presenta.


Assuntos
Embolia Aérea , Embolia Intracraniana , Doenças Pulmonares Intersticiais , Humanos , Masculino , Embolia Aérea/etiologia , Embolia Aérea/diagnóstico por imagem , Embolia Aérea/terapia , Idoso , Evolução Fatal , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/complicações , Embolia Intracraniana/etiologia , Embolia Intracraniana/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Oxigenoterapia Hiperbárica
14.
Medicine (Baltimore) ; 103(26): e38618, 2024 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-38941435

RESUMO

RATIONALE: Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare but serious complication in patients with malignancy; its main manifestation includes acute pulmonary hypertension with severe respiratory distress. More than 200 cases have been reported since it was first identified in 1990. PTTM accounts for approximately 0.9% to 3.3% of deaths due to malignancy, but only a minority of patients are diagnosed ante-mortem, with most patients having a definitive diagnosis after autopsy. PATIENT CONCERNS: Two middle-aged women both died within a short period of time due to progressive dyspnea and severe pulmonary hypertension. DIAGNOSES: One patient was definitively confirmed as a gastrointestinal malignant tumor by liver puncture biopsy pathology. Ultimately, the clinical diagnosis was pulmonary tumor thrombotic microangiopathy. INTERVENTIONS: The patient was treated symptomatically with oxygen, diuresis, and anticoagulation, while a liver puncture was perfected to clarify the cause. OUTCOMES: Two cases of middle-aged female patients with rapidly progressive pulmonary hypertension and respiratory failure resulted in death with malignant neoplasm. LESSONS: PTTM has a rapid onset and a high morbidity and mortality rate. Our clinicians need to be more aware of the need for timely diagnosis through a targeted clinical approach, leading to more targeted treatment and a better prognosis.


Assuntos
Microangiopatias Trombóticas , Humanos , Feminino , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/diagnóstico , Pessoa de Meia-Idade , Evolução Fatal , Hipertensão Pulmonar/etiologia , Neoplasias Gastrointestinais/complicações , Neoplasias Gastrointestinais/patologia , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/diagnóstico
15.
Diagn Pathol ; 19(1): 89, 2024 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-38943126

RESUMO

BACKGROUND: Waldenström's macroglobulinemia (WM) is defined as a lymphoplasmacytic lymphoma (LPL) involving the bone marrow (BM) with presence of IgM monoclonal protein, and comprises > 95% of all LPL cases. Rituximab-based regimens have been predominant in the management of WM. Infusion-related reactions (IRRs) are a primary concern with rituximab, although it is generally better tolerated with less toxicity than conventional anticancer agents. Here, we present an autopsy case of an elderly man who died suddenly after receiving the initial infusion of rituximab for WM/LPL. CASE PRESENTATION: An 84-year-old man was found dead in his bedroom. He had undergone the initial intravenous rituximab infusion for progressive anemia related to Waldenström's macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) approximately 15 h before death. Although the protocol for rituximab administration and additional medication was considered appropriate, he exhibited several symptoms consistent with infusion-related reactions (IRRs) during the infusion. Autopsy revealed monotonous proliferation of small-to-medium-sized lymphocytic cells in the bone marrow, consistent with the premortem diagnosis of WM/LPL. Additionally, immunoglobulin λ-light chain-derived amyloid (ALλ) deposition was identified in all organs other than the brain. Although ALλ deposition and LPL infiltration were found in the heart, they were not severe enough to cause severe functional impairment. Severe congestion and/or edema were observed in the lungs, liver, and brain. Although significant inflammatory cell infiltration was not found in any organs, laboratory tests revealed elevated serum levels of inflammatory cytokines, including interleukin-1ß, interleukin-6, tumor necrosis factor-α and the presence of IgM-λ monoclonal protein. CONCLUSION: Acute IRRs associated with the initial rituximab infusion were the major contributing factor to his sudden unexpected death. The autopsy findings of present case suggest the necessity for thorough monitoring of older patients with WM/LPL undergoing rituximab treatment, particularly when pronounced IRRs occur during the first administration, in addition to investigating complications of WM/LPL before infusion.


Assuntos
Autopsia , Rituximab , Macroglobulinemia de Waldenstrom , Humanos , Macroglobulinemia de Waldenstrom/tratamento farmacológico , Macroglobulinemia de Waldenstrom/patologia , Macroglobulinemia de Waldenstrom/complicações , Rituximab/efeitos adversos , Rituximab/administração & dosagem , Masculino , Idoso de 80 Anos ou mais , Morte Súbita/etiologia , Antineoplásicos Imunológicos/efeitos adversos , Antineoplásicos Imunológicos/administração & dosagem , Medula Óssea/patologia , Evolução Fatal , Infusões Intravenosas
16.
BMJ Case Rep ; 17(6)2024 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-38945554

RESUMO

Extranodal involvement in diffuse large B-cell lymphoma (DLBCL) is defined as disease outside of the lymph nodes and occurs in up to one-third of patients, though multiorgan extranodal involvement is rare. Here, we describe a case of a patient presenting with widely metastatic lesions, including involvement of the lung, parotid gland, breast, pancreas, femur and multiple soft tissue masses, with initial concern for primary breast malignancy. Breast pathology and imaging were consistent with triple-expressor, double-hit stage IV high-grade B-cell lymphoma with extensive extranodal involvement. Extranodal involvement is a poor prognostic factor associated with high rates of treatment failure, and novel therapies targeting CD19 are currently being studied for relapsed and refractory DLBCL. Extranodal disease is a complex entity that can involve virtually any organ system and should be considered for new presentations of malignancy.


Assuntos
Linfoma Difuso de Grandes Células B , Humanos , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Feminino , Pessoa de Meia-Idade , Neoplasias da Mama/patologia , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/secundário , Neoplasias Parotídeas/patologia , Neoplasias Parotídeas/diagnóstico , Neoplasias Parotídeas/secundário , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/diagnóstico por imagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Evolução Fatal
17.
BMC Infect Dis ; 24(1): 645, 2024 Jun 27.
Artigo em Inglês | MEDLINE | ID: mdl-38937714

RESUMO

BACKGROUND: Tuberculosis (TB), one of the leading causes of death worldwide, has a higher incidence among indigenous people. Albeit uncommon, autoimmune hemolytic anemia (AIHA) has been deemed a risk condition to develop mycobacterial infection, as a result of the immunosuppressive treatments. TB, in turn, can be a predisposing factor for secondary infections. CASE PRESENTATION: Here we present a case of a 28-year-old indigenous woman from Colombia, previously diagnosed with AIHA and pulmonary TB. Despite various treatments, therapies and medical interventions, the patient died after severe medullary aplasia of multiple causes, including secondary myelotoxicity by immunosuppressive therapy and secondary disseminated infections, underlining infection by Staphylococcus aureus, Klebsiella pneumoniae and Candida glabrata, which were identified as drug-resistant microorganisms. Together, this led to significant clinical complications. Invasive aspergillosis was diagnosed at autopsy. CONCLUSIONS: This report presents a rarely finding of AIHA followed by TB, and highlights the great challenges of dealing with co-infections, particularly by drug resistant pathogens. It also aims to spur governments and public health authorities to focus attention in the prevention, screening and management of TB, especially among vulnerable communities, such as indigenous people.


Assuntos
Anemia Hemolítica Autoimune , Coinfecção , Humanos , Feminino , Adulto , Coinfecção/microbiologia , Evolução Fatal , Anemia Hemolítica Autoimune/complicações , Colômbia , Klebsiella pneumoniae/isolamento & purificação , Staphylococcus aureus/isolamento & purificação , Candida glabrata/isolamento & purificação , Tuberculose Pulmonar/tratamento farmacológico , Tuberculose Pulmonar/complicações , Tuberculose Pulmonar/microbiologia , Infecções Estafilocócicas/microbiologia , Povos Indígenas , Candidíase/tratamento farmacológico , Candidíase/microbiologia
18.
Medicina (Kaunas) ; 60(6)2024 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-38929494

RESUMO

Introduction: Signet-ring cells are typically associated with mucin-secreting epithelium; thus, they are most commonly found in the gastrointestinal tract, but not exclusively. Primary signet-ring cell carcinoma of the prostate is a rare and poorly differentiated, aggressive acinar adenocarcinoma variant with a grim prognosis. Clinical Case: In June of 2023, a 54-year-old Caucasian male presented with a complaint of lower urinary tract obstructive symptoms with occasional macrohematuria, non-specific body aches, and shortness of breath. A prostate specimen obtained in transurethral resection of the prostate was sent for histopathological examination. After a series of extraprostatic diagnostic workups, including fibrogastroduodenoscopy, colonoscopy computed tomography imaging, and immunohistochemical studies, the patient was diagnosed with primary prostatic signet-ring cell adenocarcinoma stage IV. Unfortunately, due to the advanced stage of the disease, PE, and third-degree thrombocytopenia, the patient was not a candidate for chemotherapy and died of cardiopulmonary insufficiency later that week. Discussion: Prostatic signet-ring cell carcinoma accounts for 0.02% of all prostate adenocarcinoma cases. Due to its nature and epidemiology, a diligent extraprostatic investigation has to be carried out. The disease often presents with unremarkable clinical symptoms and variable serum prostate-specific antigen results, which may contribute to its late diagnosis. Inconsistent immunohistochemical findings and an unpredictable response to hormonal treatment together pose both diagnostic and therapeutic challenges that negatively affect the prognosis. Conclusions: This study highlights the importance of a multidisciplinary approach and the need for diagnostic and therapeutic consensus within the research community in search of the primary site of the disease, which may positively influence the prognosis.


Assuntos
Carcinoma de Células em Anel de Sinete , Mucinas , Neoplasias da Próstata , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias da Próstata/patologia , Neoplasias da Próstata/diagnóstico , Carcinoma de Células em Anel de Sinete/diagnóstico , Carcinoma de Células em Anel de Sinete/patologia , Mucinas/análise , Adenocarcinoma/diagnóstico , Evolução Fatal
19.
Medicina (Kaunas) ; 60(6)2024 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-38929502

RESUMO

Introduction: Renal haemangioma is a benign tumour, and due to its characteristics, it must be distinguished from malignant diseases. We present a clinical case of primary renal angiosarcoma initially mistaken for haemangioma due to their similarity. Case report: A 58-year-old man was admitted to the hospital with suspicion of pulmonary embolism. The patient complained of pain on the left side. An ultrasound and CT scan of the abdomen showed a tumour mass ~20.5 × 17.2 × 15.4 cm in size in the projection of the left kidney. On CT images, there were data for clear cell renal clear cell carcinoma (ccRCC). A left nephrectomy was performed. However, histological examination revealed renal haemangioma. Three months later, the patient presented to the hospital with abdominal and lumbar pain. A CT scan showed multiple small hypoechoic foci up to 2 cm in size in the liver, lungs, and intra-abdominally, with the most data for carcinosis. Histological re-verification of the left kidney showed a renal vascular tumour with pronounced signs of infarction and necrosis with the majority of the evidence supporting angiosarcoma. Despite treatment, the patient's outcome was fatal. Conclusions: Based on the clinical presentation, radiological images and histological examination data, the tumour was initially misdiagnosed as kidney haemangioma. Due to the rarity of this tumour, there are no established treatment protocols or clinical guidelines for managing primary kidney angiosarcoma.


Assuntos
Hemangiossarcoma , Neoplasias Renais , Humanos , Masculino , Pessoa de Meia-Idade , Hemangiossarcoma/diagnóstico , Neoplasias Renais/patologia , Neoplasias Renais/diagnóstico , Neoplasias Renais/diagnóstico por imagem , Evolução Fatal , Tomografia Computadorizada por Raios X , Nefrectomia
20.
Medicina (Kaunas) ; 60(6)2024 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-38929623

RESUMO

Background and Objectives: Prolidase deficiency (PD) is a rare, life-threatening, genetically determined disease with an incidence of 1-2 cases per 1 million births. The disease inhibits collagen synthesis, which leads to organ and systems failure, including hepato- and splenomegaly, immune disorders, chronic ulcerative wounds, respiratory infections, and pulmonary fibrosis. The complexity of the problems associated with this disease necessitates a comprehensive approach and the involvement of an interdisciplinary team. The objective was to present the treatment and care plan, as well as complications of PD, in a young woman following admission to an intensive care unit (ICU). Materials and Methods: A retrospective observational single-case study. Results: A 26-year-old woman with PD was hospitalized in the ICU for acute respiratory failure. The presence of difficult-to-heal extensive leg ulcers and the patient's immunocompromised condition resulted in the development of sepsis with multiple organ failure (respiratory and circulatory, liver and kidney failure). Complex specialized treatment consisting of wound preparation, limb amputation, the minimization of neuropathic pain, mechanical ventilation, renal replacement therapy, circulatory stabilization, and the prevention of complications of the disease and of therapy were applied. On the 83rd day of hospitalization, the patient expired. Conclusions: Despite the use of complex treatment and care, due to the advanced nature of the disease and the lack of therapies with proven efficacy, treatment was unsuccessful. There is a need for evidence-based research to develop effective treatment guidelines for PD.


Assuntos
Unidades de Terapia Intensiva , Insuficiência de Múltiplos Órgãos , Deficiência de Prolidase , Sepse , Humanos , Feminino , Adulto , Insuficiência de Múltiplos Órgãos/etiologia , Sepse/complicações , Deficiência de Prolidase/complicações , Estudos Retrospectivos , Evolução Fatal
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