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Berentsen S. Cold-antibody Autoimmune Hemolytic Anemia: its Association with Neoplastic Disease and Impact on Therapy. Curr Oncol Rep 2024; 26:1085-1096. [PMID: 38874820 DOI: 10.1007/s11912-024-01569-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/05/2024] [Indexed: 06/15/2024]
Abstract
PURPOSE OF REVIEW Cold-antibody mediated autoimmune hemolytic anemia (cAIHA) is subclassified as cold agglutinin disease (CAD), secondary cold agglutinin syndrome (CAS), and paroxysmal cold hemoglobinuria (PCH). This review aims to address the occurrence of neoplastic disorders with these three entities and analyze the impact of such neoplasias on treatment for cAIHA. RECENT FINDINGS "Primary" CAD is a distinct clonal B-cell lymphoproliferative disorder in probably all cases, although not classified as a malignant lymphoma. CAS is secondary to malignant lymphoma in a minority of cases. Recent findings allow a further clarification of these differential diagnoses and the therapeutic consequences of specific neoplastic entities. Appropriate diagnostic workup is critical for therapy in cAIHA. Patients with CAD should be treated if they have symptomatic anemia, significant fatigue, or bothersome circulatory symptoms. The distinction between CAD and CAS and the presence of any underlying malignancy in CAS have essential therapeutic implications.
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Affiliation(s)
- Sigbjørn Berentsen
- Department of Research and Innovation, Haugesund Hospital, Helse Fonna Hospital Trust, Haugesund, Norway.
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Alshareefy Y, Shen CY, Prekash RJ. Exploring the molecular pathogenesis, diagnosis and treatment of fibrolamellar hepatocellular carcinoma: A state of art review of the current literature. Pathol Res Pract 2023; 248:154655. [PMID: 37429175 DOI: 10.1016/j.prp.2023.154655] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/13/2023] [Accepted: 06/25/2023] [Indexed: 07/12/2023]
Abstract
This paper aims to present a detailed overview of fibrolamellar carcinoma (FLC), a variant of hepatocellular carcinoma (HCC) that accounts for approximately 1-9% of all cases a. according to the SEER database. Despite ongoing research, the aetiology of FLC tumours remains unclear. Nevertheless, FLC is believed to have a better overall prognosis than other primary liver tumours, such as hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma. This study aims to present a comprehensive overview of fibrolamellar carcinoma (FLC), with a focus on its epidemiology, pathogenesis, diagnosis, treatment, and prognosis. FLC frequently incorporate features of stomach pain, weight loss, and malaise in their clinical signs and symptoms, which are generally nonspecific Ultimately, the most common physical finding is an abdominal mass or hepatomegaly. With this said, several unusual presentations have been documented such as Budd Chiari syndrome, severe anaemia, non-bacterial thrombotic endocarditis and many more. In regards to this tumour's genetic analysis, it is characterised by a 400 kb deletion on chromosome 19 leading to a functional DNAJB1-PRKACA chimeric transcript in addition to tetraploidy in 50% of cases. FLC is chromosomally stable as compared to typical HCC. mTOR pathway activation has also been found to play a critical role in 47% of these tumours and EFGR over-expression is also evident. Fibrolamellar carcinomas (FLCs) exhibit a distinctive gross appearance, characterized by a yellow to pale tan colour, with a consistency that can vary from soft to firm and hard. In addition, a central scar is observed in 60-70% of FLC cases. The central scar is typically white or grey in colour and has a fibrous appearance, which is often surrounded by nodular, tumour-like tissue. Its histologic appearance is characterized by large polygonal cells with abundant eosinophilic cytoplasm, large vesiculated nuclei, large nucleoli, and arranged in lamellar bands of collagen fibres. Lamellar bands of fibrosis, consisting of collagen type I, III and IV, have also been identified as a distinctive histologic feature that is observed under low power magnification. Ultrasound, CT and MRI along with image guided biopsy are the primary modalities in diagnosis. Current management options include systemic therapy which has thus far been unremarkable with platinum-based therapies as well combination therapy with interferon alpha-2b being the most successful options. Surgical resection remains the primary treatment modality and there have been no advances in targeted therapies. Although the prognosis for FLC is favourable as compared to other hepatic cancer subtypes such as intrahepatic cholangiocarcinoma, there is a high rate of recurrence ranging from 33% to 100% with a median recurrence-free survival of 20-48 months. As a result of this there is a low overall cure rate associated with this tumour type and much more research is required to gain an in-depth understanding of the molecular mechanisms occurring in order to provide more adequate treatment to patients who suffer from this condition.
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Affiliation(s)
- Yasir Alshareefy
- School of Medicine, Trinity College Dublin, University of Dublin, Ireland.
| | - Chai Yu Shen
- Department of Medicine, Manipal University College, Malaysia
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Alsadery HA, Almaiman H, Alibrah R, Mnayan M, Alblowi A, Alzayyat R, Alshahrani A. Case Report of Fibrolamellar Hepatocellular Carcinoma in A 15-Year-old Male. Med Arch 2022; 76:387-390. [PMID: 36545449 PMCID: PMC9760234 DOI: 10.5455/medarh.2022.76.387-390] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2022] [Accepted: 10/22/2022] [Indexed: 11/16/2022] Open
Abstract
Background A rare form of hepatocellular cancer is called fibrolamellar hepatocellular carcinoma (FL-HCC) which occurs mostly in young adults who are medically free, regardless of their gender. It usually presents with abdominal pain with right upper quadrant palpable mass, nausea, and weight loss associated with higher Alpha-Fetoprotein (AFP) in some cases. Objective We report a case of a 15-year-old male patient who was diagnosed with (FL-HCC), successfully treated with surgical resection and is currently free of relapses. Case presentation A 15-year-old male patient with no previous medical or surgical history, presented with recurrent vomiting for two months, weight loss, and loss of appetite. Patient presented with normal systemic examination except for abdominal examination which revealed a generalized distended abdomen with mild tenderness in the right upper quadrant with the presence of hepatomegaly. Laboratory and radiological investigation showed high level of (AFP). CT and liver MRI showed large right hepatic lobe lesion then TRU-CUT needle biopsy was performed which showed Fibrolamellar hepatocellular carcinoma and patient underwent surgical resection with no postoperative complication followed by multiple cycle of chemotherapy and no signs of relapse with 3 year follow up. Conclusion Fibrolamellar hepatocellular carcinoma is rear type hepatocellular carcinoma which occurs mostly in young adults who are medically free with vague symptom and to diagnose it need high index of suspicion and variers Laboratory and radiological investigation including biopsy. However, it can be treated successfully by surgical resection followed by chemotherapy in selected cases if diagnosis in timely manner.
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Affiliation(s)
- Humood Ahmed Alsadery
- Department of General Surgery, King Fahad Hospital of the University, Imam Abdulrahman Bin Faisal University, College of Medicine, Dammam, Saudi Arabia
| | - Hisham Almaiman
- Department of Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
| | - Rawan Alibrah
- Department of Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
| | - Marooh Mnayan
- Department of Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
| | - Abdulrahman Alblowi
- Department of General Surgery, King Fahad Hospital of the University, Imam Abdulrahman Bin Faisal University, College of Medicine, Dammam, Saudi Arabia
| | - Remah Alzayyat
- Department of General Surgery, King Fahad Hospital of the University, Imam Abdulrahman Bin Faisal University, College of Medicine, Dammam, Saudi Arabia
| | - Abdulwahab Alshahrani
- Department of General Surgery, King Fahad Hospital of the University, Imam Abdulrahman Bin Faisal University, College of Medicine, Dammam, Saudi Arabia,Department of Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
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Abdelhamed W, El-Kassas M. Fibrolamellar hepatocellular carcinoma: A rare but unpleasant event. World J Gastrointest Oncol 2022; 14:1103-1114. [PMID: 35949219 PMCID: PMC9244987 DOI: 10.4251/wjgo.v14.i6.1103] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/15/2022] [Revised: 03/19/2022] [Accepted: 05/08/2022] [Indexed: 02/06/2023] Open
Abstract
Fibrolamellar carcinoma (FLC) is a rare variant of hepatocellular carcinoma (HCC), comprising 1%-9% of all HCCs. FLC is a poorly understood malignancy, which seems to be more prevalent in young patients with no underlying liver diseases. The term "fibrolamellar" is derived from thick fibrous collagen bands surrounding the tumor cells. Unlike HCC, cirrhosis and viral hepatitis infection are not predisposing to FLC, and it is not associated with elevations in serum alpha-fetoprotein. FLC patients often present with vague abdominal pain, nausea, malaise, and weight loss. Most cases present are at an advanced stage at the time of initial diagnosis. However, curative treatment options can still be offered to up to 70% of patients. Surgery (resection/liver transplantation) is the mainstay of treatment and the only potentially curative option. FLCs have been less chemo-responsive than the conventional HCC, however, in advanced cases, multimodality treatments can be effective. Recent advances in molecular studies of FLC have found a unique DNAJB1-PRKACA fusion transcript in most of the cases studied. The review aims to describe clinical characteristics, diagnostic methods, and therapeutic modalities for this rare tumor to raise awareness among clinicians and surgeons.
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Affiliation(s)
- Walaa Abdelhamed
- Department of Endemic Medicine, Sohag University, Sohag 14322, Egypt
| | - Mohamed El-Kassas
- Department of Endemic Medicine, Faculty of Medicine, Helwan University, Cairo 11795, Egypt
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Unusual co-occurrence of cold agglutinin disease in a bladder transitional cell carcinoma patient with unexplained anemia and cold-related symptoms. Chin Med J (Engl) 2020; 134:608-610. [PMID: 33323815 PMCID: PMC7929532 DOI: 10.1097/cm9.0000000000001277] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
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Lemekhova A, Hornuss D, Polychronidis G, Mayer P, Rupp C, Longerich T, Weiss KH, Büchler M, Mehrabi A, Hoffmann K. Clinical features and surgical outcomes of fibrolamellar hepatocellular carcinoma: retrospective analysis of a single-center experience. World J Surg Oncol 2020; 18:93. [PMID: 32397993 PMCID: PMC7218513 DOI: 10.1186/s12957-020-01855-2] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/26/2019] [Accepted: 04/16/2020] [Indexed: 02/06/2023] Open
Abstract
Background Clinicopathological features and surgical outcomes of patients with fibrolamellar hepatocellular carcinoma (FL-HCC) are underreported. The aim of this study is to describe clinical characteristics and surgical outcomes for patients with this rare tumor to raise awareness among clinicians and surgeons. Methods Retrospective review of records of a tertiary referral center and specialized liver unit was performed. Out of 3623 patients who underwent liver resection, 366 patients received surgical treatment for HCC; of them, eight (2.2%) had FL-HCC and were resected between October 2001 and December 2018. Results Eight patients (3 males and 5 females) with FL-HCC (median age 26 years) underwent primary surgical treatment. All patients presented with unspecific symptoms or were diagnosed as incidental finding. No patient had cirrhosis or other underlying liver diseases. Coincidentally, three patients (37.5%) had a thromboembolic event prior to admission. The majority of patients had BCLC stage C and UICC stage IIIB/IVA; four patients (50%) presented with lymph node metastases. The median follow-up period was 33.5 months. The 1-year survival was 71.4%, and 3-year survival was 57.1%. Median survival was at 36.4 months. Five patients (62.5%) developed recurrent disease after a median disease-free survival of 9 months. Two patients (25.0%) received re-resection. Conclusion FL-HCC is a rare differential diagnosis of liver masses in young patients. Since the prognosis is limited, patients with incidental liver tumors or lesions with suspicious features in an otherwise healthy liver should be presented at a specialized hepatobiliary unit. Thromboembolism might be an early paraneoplastic symptom and needs to be elucidated further in the context of FL-HCC.
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Affiliation(s)
- Anastasia Lemekhova
- Department of General, Visceral, and Transplantation Surgery, Ruprecht Karls University Hospital, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany.,Liver Cancer Centre Heidelberg (LCCH), Heidelberg, Germany
| | - Daniel Hornuss
- Liver Cancer Centre Heidelberg (LCCH), Heidelberg, Germany.,Department of Gastroenterology and Hepatology, Ruprecht Karls University Hospital, Heidelberg, Germany
| | - Georgios Polychronidis
- Department of General, Visceral, and Transplantation Surgery, Ruprecht Karls University Hospital, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany.,Liver Cancer Centre Heidelberg (LCCH), Heidelberg, Germany
| | - Philipp Mayer
- Liver Cancer Centre Heidelberg (LCCH), Heidelberg, Germany.,Department of Diagnostic and Interventional Radiology, Ruprecht Karls University Hospital, Heidelberg, Germany
| | - Christian Rupp
- Department of Gastroenterology and Hepatology, Ruprecht Karls University Hospital, Heidelberg, Germany
| | - Thomas Longerich
- Liver Cancer Centre Heidelberg (LCCH), Heidelberg, Germany.,Institute of Pathology, University Hospital Heidelberg, Heidelberg, Germany
| | - Karl-Heinz Weiss
- Liver Cancer Centre Heidelberg (LCCH), Heidelberg, Germany.,Department of Gastroenterology and Hepatology, Ruprecht Karls University Hospital, Heidelberg, Germany
| | - Markus Büchler
- Department of General, Visceral, and Transplantation Surgery, Ruprecht Karls University Hospital, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany.,Liver Cancer Centre Heidelberg (LCCH), Heidelberg, Germany
| | - Arianeb Mehrabi
- Department of General, Visceral, and Transplantation Surgery, Ruprecht Karls University Hospital, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany.,Liver Cancer Centre Heidelberg (LCCH), Heidelberg, Germany
| | - Katrin Hoffmann
- Department of General, Visceral, and Transplantation Surgery, Ruprecht Karls University Hospital, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany. .,Liver Cancer Centre Heidelberg (LCCH), Heidelberg, Germany.
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Tauseef A, Asghar MS, Zafar M, Ahmed I, Dawood M, Shaikh T, Khan N, Alam T. Cold autoimmune hemolytic anemia: a rare association with triple-positive breast cancer. J Community Hosp Intern Med Perspect 2020; 9:499-502. [PMID: 32002158 PMCID: PMC6968714 DOI: 10.1080/20009666.2019.1698262] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/05/2019] [Accepted: 09/18/2019] [Indexed: 11/16/2022] Open
Abstract
A 45-year-old Asian woman was presented with fever, easy fatigability, shortness of breath, cervical and axillary lymphadenopathy and other signs and symptoms of anemia. After all the baseline work-up, the patient was investigated for Mono-coombs C3d levels, which were elevated, suggesting the diagnosis of Cold autoimmune hemolytic anemia (Cold AIHA). An Ultrasound-guided true-cut biopsy was done to determine the primary cause associated with it, which showed the presence of tumor cells arranged in cords and clusters. They have dark staining cells with mitotic activity, suggestive of breast carcinoma as an association of Cold AIHA. Estrogen receptor (ER), progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER2) were sent, which came out to be positive. So, the patient was diagnosed with Cold AIHA in association with triple-positive breast cancer.
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Affiliation(s)
- Abubakar Tauseef
- Resident Physician in Internal Medicine Department, Dow University Hospital, Karachi, Pakistan
| | - Muhammad Sohaib Asghar
- Resident Physician in Internal Medicine Department, Dow University Hospital, Karachi, Pakistan
| | - Maryam Zafar
- Resident Physician in Internal Medicine Department, Dow University Hospital, Karachi, Pakistan
| | - Iftekhar Ahmed
- Resident Physician in Internal Medicine Department, Dow University Hospital, Karachi, Pakistan
| | | | - Tooba Shaikh
- Resident Physician in Internal Medicine Department, Dow University Hospital, Karachi, Pakistan
| | - Narmin Khan
- Resident Physician in Internal Medicine Department, Dow University Hospital, Karachi, Pakistan
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Johnstone P, Khan O. Pembrolizumab-associated autoimmune haemolytic anaemia. BMJ Case Rep 2019; 12:e229064. [PMID: 31653618 PMCID: PMC6827724 DOI: 10.1136/bcr-2018-229064] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/19/2019] [Indexed: 11/04/2022] Open
Abstract
Treatment paradigms have recently changed with the introduction of immunotherapy; autoimmune toxicities that can arise are frequently very different from the more familiar chemotherapy toxicities. We present a clinical case of autoimmune haemolytic anaemia (AIHA) secondary to pembrolizumab occurring in a 73-year-old male patient being treated for lung adenocarcinoma, who had received 13 cycles of pembrolizumab. Treatment was immediately stopped and he was treated with high dose steroids to which he responded both clinically and biochemically. There have been prior reports of immunotherapy-associated AIHA with the use of cytotoxic T-lymphocyte-associated antigen-4 inhibitors, such as ipilimumab, but very few reports of programmed death-1 (PD-1)/programmed death-ligand 1 (PDL-1) inhibitor associated AIHA. We highlight a rare case of AIHA as an adverse effect of pembrolizumab, a PD-1 inhibitor. Although unusual, it is important to be vigilant for haematological immune-related adverse events.
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Affiliation(s)
| | - Omar Khan
- Oncology, Great Western Hospitals NHS Foundation Trust, Swindon, UK
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Lafaro KJ, Pawlik TM. Fibrolamellar hepatocellular carcinoma: current clinical perspectives. J Hepatocell Carcinoma 2015; 2:151-7. [PMID: 27508204 PMCID: PMC4918295 DOI: 10.2147/jhc.s75153] [Citation(s) in RCA: 30] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
Fibrolamellar carcinoma (FLC) is a variant of hepatocellular carcinoma (HCC), which comprises ∼1%–9% of all HCCs. Although FLC is a variant of HCC, it is distinct from HCC in that it most often affects younger patients (10–35 years of age) with no underlying liver disease. FLC often presents with vague abdominal pain, nausea, abdominal fullness, malaise, and weight loss. Surgery is the current mainstay of treatment for FLC and remains the only potentially curative option. While FLCs are considered less responsive to chemotherapy than their classic HCC counterparts, there have been suggestions that multimodality treatments may be effective, especially in advanced cases. Further research is necessary to determine effective systemic therapies as an adjunct to surgery for FLC.
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Affiliation(s)
- Kelly J Lafaro
- Center for Pancreatic Cancer Research, Memorial Sloan Kettering Cancer Center, New York, NY
| | - Timothy M Pawlik
- Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
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10
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Chellappan A, Bammigatti C, Palamalai S. An unusual presentation of metastatic breast carcinoma as cold autoimmune hemolytic anemia. Turk J Haematol 2015; 32:85-6. [PMID: 25805683 PMCID: PMC4439915 DOI: 10.4274/tjh.2014.0373] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022] Open
Affiliation(s)
- Anand Chellappan
- Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Department of Internal Medicine, Puducherry, India. E-mail:
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Del Proposto G, Antonelli M, Cerrone P, Bruno A, Costantino L, Ricciardi E, Rossi P, Modica S, Adorno G. Cancer of the sigmoid colon and antibodies. A puzzle. Transfus Apher Sci 2015; 52:220-1. [PMID: 25578649 DOI: 10.1016/j.transci.2014.12.025] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2014] [Accepted: 12/16/2014] [Indexed: 11/24/2022]
Abstract
UNLABELLED In this work we describe the case report of a woman affected by cancer of the sigmoid colon and with a positive direct antiglobulin test (DAT) and indirect antiglobulin test (IAT). Case report with results: A meticulous medical history showed that the woman had been suffering from recurrent fetal loss. Then she had cardiac and coagulative problems. These data suggested a phospholipid syndrome. CONCLUSION The patient had a medical history positive for a phospholipid syndrome and we think that this disease could explain the onset of the autoantibody.
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Affiliation(s)
| | | | | | - Antoine Bruno
- Hematology Section, Tor Vergata University, Rome, Italy
| | | | | | - Piero Rossi
- General Surgery Section, Tor Vergata University, Rome, Italy
| | - Stella Modica
- Internal Medicine, Tor Vergata University, Rome, Italy
| | - Gaspare Adorno
- SIMT Tor Vergata Hospital, Immunohematology Section, Tor Vergata University, Rome, Italy
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Abstract
AbstractCold agglutinin disease is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia. We reviewed the clinical and pathologic features, prognosis, and management in the literature and describe our institutional experience to improve strategies for accurate diagnosis and treatment. Retrospective analysis identified 89 patients from our institution with cold agglutinin disease from 1970 through 2012. Median age at symptom onset was 65 years (range, 41 to 83 years), whereas the median age at diagnosis was 72 years (range, 43 to 91 years). Median survival of all patients was 10.6 years, and 68 patients (76%) were alive 5 years after the diagnosis. The most common symptom was acrocyanosis (n = 39 [44%]), and many had symptoms triggered by cold (n = 35 [39%]) or other factors (n = 20 [22%]). An underlying hematologic disorder was detected in 69 patients (78%). Thirty-six patients (40%) received transfusions during their disease course, and 82% received drug therapy. Rituximab was associated with the longest response duration (median, 24 months) and the lowest proportion of patients needing further treatment (55%). Our institution’s experience and review of the literature confirms that early diagnostic evaluation and treatment improves outcomes in cold agglutinin disease.
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Torbenson M. Fibrolamellar carcinoma: 2012 update. SCIENTIFICA 2012; 2012:743790. [PMID: 24278737 PMCID: PMC3820672 DOI: 10.6064/2012/743790] [Citation(s) in RCA: 65] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 07/26/2012] [Accepted: 08/22/2012] [Indexed: 06/02/2023]
Abstract
Fibrolamellar carcinomas are a unique type of primary liver cancer. They occur most commonly in children and young adults. Their etiology remains a mystery, as they are not associated with chronic liver disease. Fibrolamellar carcinomas are not indolent tumors, but have an overall better prognosis than typical hepatocellular carcinomas, in part because of the younger age at presentation and the lack of cirrhosis. The most important prognostic feature is whether the tumor is resectable. Histologically, the tumor is made up of large cells that contain abundant mitochondria. The nuclei of the tumor cells have prominent nucleoli. The tumor cells induce the formation of extensive intratumoral fibrosis, which often grows in parallel, or lamellar bands. The tumor cells clearly show hepatocellular features but are also unique in showing both biliary and neuroendocrine differentiation. The uniqueness of fibrolamellar carcinoma extends to their molecular findings. While the genetic abnormalities that lead to fibrolamellar carcinomas are not yet known, studies have shown that they lack mutations in the genes most commonly mutated in typical hepatocellular carcinoma (TP53 and CTNNB1). In this paper, the clinical, pathological, and basic science literature on fibrolamellar carcinoma is comprehensively reviewed. Key areas of needed research are also discussed.
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Affiliation(s)
- Michael Torbenson
- Department of Pathology, The Johns Hopkins University School of Medicine, Room B314, 1503 E. Jefferson, Bond Street Building, Baltimore, MD 21231, USA
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Berentsen S, Tjønnfjord GE. Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia. Blood Rev 2012; 26:107-15. [DOI: 10.1016/j.blre.2012.01.002] [Citation(s) in RCA: 114] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
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Dedeepiya VD, Terunuma H, Deng X, Baskar S, Manjunath SR, Senthilkumar R, Murugan P, Thamaraikannan P, Srinivasan T, Preethy S, Abraham SJK. A comparative analysis of in vitro expansion of natural killer cells of a patient with autoimmune haemolytic anaemia and ovarian cancer with patients with other solid tumours. Oncol Lett 2011; 3:435-440. [PMID: 22740927 DOI: 10.3892/ol.2011.498] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2011] [Accepted: 11/21/2011] [Indexed: 11/06/2022] Open
Abstract
The functional profile of natural killer (NK) cells has been reported to be lower in auto-immune haemolytic anaemia (AIHA). In this study, we report a comparative analysis of peripheral blood mononuclear cells (PBMNCs) and the in vitro expansion of NK cells in a patient with AIHA and cancer, with that of other cancer patients without AIHA. PBMNCs and in vitro NK-cell expansion of a 64-year old female patient with ovarian cancer and AIHA was compared with that of four other patients with cancer without AIHA who underwent autologous immune enhancement therapy (AIET). The NK cells were cultured using autologous plasma without feeder layers. The quantities of PBMNCs, NK cells and CD3-CD56+ cells were compared. The average quantity of PBMNCs per ml in Cases I to V were 10.71, 39.2, 49.26, 65.16 and 49.33×10(4), respectively, and the average maximum count of NK cells was 3.9, 1730.03, 1824.16, 1058.61 and 761×10(6), respectively. The average percentage of CD3-CD56+ cells in Cases I to V following in vitro expansion was 1.2, 65.7, 28.63, 65.9 and 40%, respectively. In the present study, probably the first in the literature, the in vitro expansion of NK cells was found to be significantly lower in the AIHA patient. Previously, only a lower NK-cell functional profile was reported. Further studies are required to establish the association between AIHA and NK-cell profile and in vitro expansion, and to find common antibodies between red blood cells (RBCs) and NK cells.
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Abstract
Fibrolamellar carcinoma (FL-Ca) is a primary malignant liver tumor at unknown etiology, without cirrhosis and usually without an increase at tumor markers, which occurs mainly in young patients. As it can simulate malignant and benign tumors, particularly FNH, diagnosis is difficult. Ultrasound and angiography show mostly uncharacteristic features. The highest specificity has CT, if calcifications are present, because these calcifications in a tumor similar to FNH are pathognomonic for FL-Ca. In MRI the central scars of FL-Ca and FNH have a different signal intensity in T2-weighted images, so that MRI becomes more and more important in the differential diagnosis to FNH.
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