Article
Medicine, General & Internal
Shelina Malla, Tushar Pawar, Biswaraj Tharu, Sijan Basnet, Andrew C. Rettew, Daniel A. Forman
Summary: Idiopathic systemic capillary leak syndrome (SCLS) is a disease characterized by increased capillary hyperpermeability, leading to hemoconcentration, hypoproteinemia, and hypovolemia. Patients commonly present with symptoms such as decreased blood volume, low blood pressure, and shock. This case report describes a 50-year-old man with idiopathic SCLS who experienced various complications.
JOURNAL OF COMMUNITY HOSPITAL INTERNAL MEDICINE PERSPECTIVES
(2022)
Article
Urology & Nephrology
Hyang-yun Lee, Jungho Shin, Su-Hyun Kim, Jin Ho Hwang
Summary: This article describes a rare disease called Idiopathic Systemic Capillary Leak Syndrome (ISCLS), characterized by recurrent episodes of shock, hemoconcentration, and hypoalbuminemia. It is easily misdiagnosed, but early diagnosis and treatment can prevent recurrence and fatal complications.
Review
Medicine, General & Internal
Shotaro Naito, Hiroyuki Yamaguchi, Noboru Hagino
Summary: Systemic capillary leak syndrome (SCLS), also known as Clarkson's disease, is a rare and potentially lethal condition characterized by hypotension, hemoconcentration, and hypoalbuminemia. This report presents a case of COVID-19-associated SCLS and highlights the importance of accurate diagnosis and understanding of the disease's pathophysiology for effective management. Further research on the occurrence and outcomes of SCLS is needed.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Pediatrics
Lauren T. Maloney, Bronwyn Baz, Dia Hazra
Summary: HLH is a rare disorder characterized by immune overactivation, with varied clinical manifestations that resemble other conditions. Diagnostic criteria and challenges in diagnosing HLH have been discussed in recent literature. A case report highlighted the importance of diagnostic flexibility in suspected HLH cases.
Article
Obstetrics & Gynecology
Nabil Belfeki, Gabrielle Postic, Nabil Abdelouahab Hadji, Tatiana Stempak Droissart, Olivier Elrodt, Faten El Hdhili, Cyrus Moini, Arsene Mekinian, Souheil Zayet
Summary: Idiopathic systemic capillary leak syndrome, a rare disease, was described in a case of a patient who presented with unusual vulvar edema. The patient received treatment and achieved a favorable outcome.
ARCHIVES OF GYNECOLOGY AND OBSTETRICS
(2022)
Article
Hematology
Amritpal Singh, Aref Al-Kali, Kebede H. Begna, Mark R. Litzow, Jeremy T. Larsen, Taimur Sher, Maymona G. Abdelmagid, Faiqa Farrukh, Kaaren K. Reichard, Naseema Gangat, Animesh Pardanani, Ayalew Tefferi
Summary: This study retrospectively examined the experience of midostaurin therapy in 33 patients with advanced systemic mastocytosis. The overall response rate was 42%, with reductions in bone marrow mast cells and normalization of serum tryptase seen in some cases. Median survival was longer in midostaurin responders, although the difference was not significant. Gastrointestinal side effects were the most common.
AMERICAN JOURNAL OF HEMATOLOGY
(2022)
Article
Allergy
Quentin Moyon, Marc Pineton de Chambrun, Marie Gousseff, Alexis Mathian, Miguel Hie, Geoffrey Urbanski, Franco Verlicchi, Stanislas Faguer, Antoine Dossier, Jean-Christophe Lega, Sophie Riviere, David Saadoun, Julie Graveleau, Marie-Josee Lucchini-Lecomte, Christine Christides, Sylvie Le Moal, Beatrice Bibes, Giuseppe Malizia, Marc Ruivard, Gilles Blaison, Laurent Alric, Christian Agard, Martin Soubrier, Jean-Francois Viallard, Herve Levesque, Georges-Etienne Rivard, Nathalie Tieulie, Arnaud Hot, Pierre-Yves Lovey, Thomas Hanslik, Francois Lhote, Vincent Eble, Jorge Alvarez Troncoso, Avinash Aujayeb, Paul Quentric, Dov Taieb, Fleur Cohen-Aubart, Marc Lambert, Zahir Amoura, EureClark Study Grp
Summary: This study aimed to evaluate the safety of tapering and withdrawal of IVIG in patients with SCLS. The results showed that IVIG withdrawal was associated with increased mortality and higher rate of recurrence in SCLS patients, while tapering IVIG may be cautiously considered in stable SCLS patients.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
(2022)
Article
Hematology
Ayalew Tefferi, Jaya Kittur, Faiqa Farrukh, Kebede H. Begna, Mrinal M. Patnaik, Aref Al-Kali, Michelle A. Elliott, Kaaren K. Reichard, Naseema Gangat, Animesh Pardanani
Summary: The study demonstrates that cladribine therapy is effective in treating both advanced and indolent/smouldering systemic mastocytosis, with acceptable adverse events. The absence of KITD816V mutation may serve as a potential marker of cladribine resistance in advanced SM, which warrants further investigation in larger studies.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Clinical Neurology
Junichiro Ohira, Hajime Yoshimura, Masashi Takanashi, Daisuke Yamashita, Shigeo Hara, Yuji Ueno, Nobutaka Hattori, Michi Kawamoto, Nobuo Kohara
Summary: This case study highlights a rare occurrence of ISCLS leading to extensive leukoencephalopathy, presenting with hemoconcentration, hypoalbuminemia, edema, and hypotension. The neurological deficits were observed in the patient after 9 years of ISCLS diagnosis, with eventual progression to coma and epileptic status. The neuropathological investigation showed perivascular plasma leakage around small vessels as the cause of leukoencephalopathy, providing valuable insights into the pathophysiology of ISCLS.
NEUROLOGICAL SCIENCES
(2021)
Article
Medicine, General & Internal
Yutaro Akiyama, Takeshi Inagaki, Shinichiro Morioka, Eiji Kusano, Norio Ohmagari
Summary: A Japanese man with a history of systemic capillary leak syndrome (SCLS) experienced exacerbation of SCLS 2 days after receiving the second dose of Pfizer-BioNTech BNT162b2 mRNA COVID-19 vaccine. After fluid therapy and albumin administration, prophylactic treatment with terbutaline and theophylline was initiated. Literature review suggests that SCLS attacks often occur 1-2 days after the second COVID-19 vaccination, therefore, patients with a history of SCLS should avoid COVID-19 vaccination and be closely monitored for 1-2 days post-vaccination.
Review
Medicine, General & Internal
E. De Tandt, D. Van Sassenbroeck, L. Heireman, J. Dierick, A. Luyckx, S. Verelst
Summary: This article describes a previously healthy 42-year-old man who was brought into the emergency department with symptoms of distributive shock. His condition rapidly deteriorated and resulted in cardiac arrest. Despite extensive measures and resuscitation efforts, the patient died a few hours later without a definitive diagnosis. Laboratory results showed severe haemoconcentration, leading to further investigations suggesting the possibility of Clarkson's disease. Emergency and intensive care physicians should be aware of this condition, which can present as unexplained refractory distributive shock with haemoconcentration and hypoalbuminemia, as it can have a fatal outcome.
ACTA CLINICA BELGICA
(2022)
Article
Medicine, General & Internal
Suman Rao, Omar Nasser, Akhila Sunkara, Rishi Singhal, Dragos Manta
Summary: Idiopathic capillary leak syndrome is a rare disorder caused by vascular membrane instability leading to protein leakage into interstitial spaces. Difficult to diagnose due to overlap with other medical emergencies. Further studies needed on the role of steroids and colloids in management.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2021)
Article
Oncology
Guillaume Joe Pegna, Min-Jung Lee, Cody J. Peer, Mehwish Ahmad, David J. Venzon, Yunkai Yu, Akira Yuno, Seth M. Steinberg, Liang Cao, William D. Figg, Renee N. Donahue, Raffit Hassan, Ira Pastan, Jane B. Trepel, Christine Alewine
Summary: The clinical trial of LMB-100 demonstrated some clinical efficacy but also revealed the potential intolerable side effect of capillary leak syndrome (CLS) when combined with nab-paclitaxel. Furthermore, LMB-100 treatment was found to cause systemic immune activation and associated inflammatory and immune changes.
Letter
Immunology
Gwang-Jun Choi, Seon Ha Baek, Junmo Kim, Jung Ho Kim, Geun-Yong Kwon, Dong Keun Kim, Yeon Haw Jung, Sejoong Kim
Summary: This report describes a case of severe adverse reaction after COVID-19 vaccination, potentially linked to the patient's history of systemic capillary leak syndrome.
EMERGING INFECTIOUS DISEASES
(2021)
Article
Medicine, General & Internal
A. Bichon, J. Carvelli, J. Bourenne, M. Gainnier, J-R Harle, N. Schleinitz
Summary: Idiopathic systemic capillary leak syndrome (ISCLS) is a sudden life-threatening condition with three consecutive phases, characterized by two clinical and biological paradoxes. Treatment for ISCLS patients focuses on symptomatic care and consideration of specific therapies when necessary.
REVUE DE MEDECINE INTERNE
(2021)
Article
Hematology
Hadiyah Y. Audil, Joselle M. Cook, Patricia T. Greipp, Prashant Kapoor, Linda B. Baughn, Angela Dispenzieri, Morie A. Gertz, Francis K. Buadi, Martha Q. Lacy, David Dingli, Amie L. Fonder, Suzanne R. Hayman, Miriam A. Hobbs, Eli Muchtar, Mustaqeem Siddiqui, Wilson Gonsalves, Yi Lisa Hwa, Nelson Leung, Yi Lin, Taxiarchis Kourelis, Rahma Warsame, Robert A. Kyle, Rhett P. Ketterling, S. Vincent Rajkumar, Shaji K. Kumar
Summary: Acquisition of 1q22 gain in multiple myeloma patients is associated with reduced overall survival. Presence of high-risk FISH abnormalities at baseline increases the risk of acquiring 1q22 gain.
AMERICAN JOURNAL OF HEMATOLOGY
(2022)
Article
Hematology
Zaid H. Abdel Rahman, Ricardo D. Parrondo, Michael G. Heckman, Mikolaj Wieczorek, Kevin C. Miller, Hassan Alkhateeb, Lisa Z. Sproat, Hemant Murthy, William J. Hogan, Mohamed A. Kharfan-Dabaja, Jess F. Peterson, Linda B. Baughn, Nicole Hoppman, Mark R. Litzow, Rhett P. Ketterling, Patricia T. Greipp, James M. Foran
Summary: Patients with therapy-related acute lymphoblastic leukaemia (tr-ALL) differed significantly from de novo ALL patients in terms of age, gender, cytogenetic abnormalities, etc. Patients with tr-ALL were less likely to achieve complete remission, but had similar overall survival rates after diagnosis and allogeneic hematopoietic cell transplantation.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Pathology
Rashmi Kanagal-Shamanna, Attilio Orazi, Robert P. Hasserjian, Daniel A. Arber, Kaaren Reichard, Eric D. Hsi, Adam Bagg, Heesun Joyce Rogers, Julia Geyer, Faezeh Darbaniyan, Kim-Anh Do, Kyle M. Devins, Olga Pozdnyakova, Tracy George, Paola Dal Cin, Patricia T. Greipp, Mark J. Routbort, Keyur Patel, Guillermo Garcia-Manero, Srdan Verstovsek, L. Jeffrey Medeiros, Sa A. Wang, Carlos Bueso-Ramos
Summary: The study revealed that patients with MDS/MPN-i(17q) were typically younger with lower platelet and neutrophil counts, commonly displayed bilobed neutrophils and had a higher mutation frequency. These patients had a poorer prognosis and shorter overall survival compared to those without i(17q).
Correction
Pathology
Rashmi Kanagal-Shamanna, Attilio Orazi, Robert P. Hasserjian, Daniel A. Arber, Kaaren Reichard, Eric D. Hsi, Adam Bagg, Heesun Joyce Rogers, Julia Geyer, Faezeh Darbaniyan, Kim-Anh Do, Kyle M. Devins, Olga Pozdnyakova, Tracy I. George, Paola Dal Cin, Patricia T. Greipp, Mark J. Routbort, Keyur Patel, Guillermo Garcia-Manero, Srdan Verstovsek, L. Jeffrey Medeiros, Sa A. Wang, Carlos Bueso-Ramos
Article
Oncology
Alaa Koleilat, Patrick W. McGarrah, Horatiu Olteanu, Daniel L. Van Dyke, James B. Smadbeck, Sarah H. Johnson, George Vasmatzis, Nicole L. Hoppman, Xinjie Xu, Rhett P. Ketterling, Patricia T. Greipp, Linda B. Baughn, Mrinal S. Patnaik, Jess F. Peterson
Summary: This case study describes a 60-year-old male patient with AML carrying a rare t(4;12)(q12;p13) chromosomal abnormality. Through conventional chromosome studies and Mate-pair sequencing, the rearrangement of ETV6 gene was confirmed, excluding the involvement of the PDGFRA gene region and influencing the patient's medical management decisions.
Article
Ophthalmology
Cristiane M. Lda, Jose Pulido, Patricia T. Greipp, Joaquin J. Garcia, Timothy W. Olsen, Lauren Dalvin, Diva Regina Salomao
Summary: BAP1 immunostaining in biopsies shows strong correlation with subsequent enucleations and well-established prognostic markers in uveal melanoma, indicating it as a potential additional prognostic tool for UM biopsies.
OCULAR ONCOLOGY AND PATHOLOGY
(2022)
Article
Hematology
Mithun Vinod Shah, Rakchha Chhetri, Ruchita Dholakia, Chung H. Kok, Naseema Gangat, Hassan B. Alkhateeb, Aref Al-Kali, Mrinal M. Patnaik, Anmol Baranwal, Patricia T. Greipp, Rong He, Kebede H. Begna, Ing Soo Tiong, Andrew H. Wei, Devendra Hiwase
Summary: Therapy-related myeloid neoplasms (t-MN) are aggressive malignancies in need of effective therapies. The BCL-2 inhibitor venetoclax represents a potential treatment option for acute myeloid leukemia, but its effectiveness in t-MN has not been extensively studied. A retrospective analysis of 378 t-MN patients revealed that the upfront use of venetoclax with other therapies improved survival, although progression-free survival (PFS) and overall survival (OS) remained poor. Therefore, further research on venetoclax should be conducted in all t-MN phenotypes.
AMERICAN JOURNAL OF HEMATOLOGY
(2022)
Article
Oncology
Alaa Koleilat, James B. Smadbeck, Cinthya J. Zepeda-Mendoza, Cynthia M. Williamson, Beth A. Pitel, Crystal L. Golden, Xinjie Xu, Patricia T. Greipp, Rhett P. Ketterling, Nicole L. Hoppman, Jess F. Peterson, Christine J. Harrison, Yassmine M. N. Akkari, Karen D. Tsuchiya, Mary Shago, Linda B. Baughn
Summary: In acute lymphoblastic leukemia, intrachromosomal amplification of chromosome 21 (iAMP21-ALL) is a common high-risk cytogenetic abnormality, and accurate identification is crucial for clinical management. The current method of identification, fluorescence in situ hybridization (FISH), has a risk of misidentification. The use of chromosomal microarray can overcome this risk effectively.
GENES CHROMOSOMES & CANCER
(2022)
Letter
Hematology
Devendra Hiwase, Christopher Hahn, Elizabeth Ngoc Hoa Tran, Rakchha Chhetri, Anmol Baranwal, Aref Al-Kali, Kirsty Sharplin, Dariusz Ladon, Rachel Hollins, Patricia Greipp, Monika Kutyna, Hassan Alkhateeb, Talha Badar, Paul Wang, David M. Ross, Deepak Singhal, Naranie Shanmuganathan, Peter Bardy, Ashanka Beligaswatte, David Yeung, Mark R. Litzow, Abhishek Mangaonkar, Pratyush Giri, Cindy Lee, Angie Yong, Noemi Horvath, Nimit Singhal, Raghu Gowda, William Hogan, Naseema Gangat, Mrinal Patnaik, Kebede Begna, Ing S. Tiong, Andrew Wei, Sharad Kumar, Anna Brown, Hamish Scott, Daniel Thomas, Chung H. Kok, Ayalew Tefferi, Mithun Vinod Shah
Letter
Hematology
Bahga Katamesh, Ahmad Nanaa, Rong He, David Viswanatha, Phuong Nguyen, Patricia Greipp, Kurt Bessonen, Naseema Gangat, Kebede Begna, Abhishek Mangaonkar, Mrinal Patnaik, William J. Hogan, Ayalew Tefferi, Mark Litzow, Mithun Vinod Shah, Cecilia Arana Yi, James Foran, Talha Badar, Hassan B. Alkhateeb, Aref Al-Kali
Letter
Oncology
Aref Al-Kali, Ahmad Nanaa, David Viswanatha, Rong He, Phuong Nguyen, Dragan Jevremovic, James M. Foran, Cecelia Arana Yi, Patricia T. Greipp, Naseema Gangat, Mrinal Patnaik, Ayalew Tefferi, Mark R. Litzow, Abhishek A. Mangaonkar, Mithun Vinod Shah, Talha Badar, Hassan B. Alkhateeb
BLOOD CANCER JOURNAL
(2023)
Article
Oncology
Nadine H. Abdallah, Alexandra N. Smith, Susan Geyer, Moritz Binder, Patricia T. Greipp, Prashant Kapoor, Angela Dispenzieri, Morie A. Gertz, Linda B. Baughn, Martha Q. Lacy, Suzanne R. Hayman, Francis K. Buadi, David Dingli, Yi L. Hwa, Yi Lin, Taxiarchis Kourelis, Rahma Warsame, Robert A. Kyle, S. Vincent Rajkumar, Shaji K. Kumar
Summary: This study aimed to estimate conditional survival (CS) at 1-8 years from diagnosis and investigate the impact of baseline prognostic factors on CS in multiple myeloma (MM) patients. A retrospective study including 2556 MM patients diagnosed between 2004 and 2019 was conducted. The findings revealed that age ≥65 and proteasome inhibitor+immunomodulatory-based induction were associated with decreased and increased survival, respectively, retained at 5 years. High-risk cytogenetic factors had a significant adverse impact at 1 and 3 years but not 5 years. Chromosome 17 abnormality was associated with decreased survival only at 1 year. Overall, the 5-year CS remained stable at 1-5 years from diagnosis among MM patients, and the prognostic impact of high-risk cytogenetic factors decreased with additional years survived.
BLOOD CANCER JOURNAL
(2023)
Article
Pathology
Nooshin K. Dashti, Judith Jebastin Thangaiah, Troy Gliem, Darlene Knutson, Sara Kloft-Nelson, Susan M. Armstrong, Ahmed Bakhshwin, Patricia Greipp, Karen J. Fritchie
Summary: Myxofibrosarcomas (MFS) are slow-growing superficial masses in elderly patients. Diagnosis can be challenging, as they can be mistaken for dedifferentiated liposarcoma with myxoid features. MDM2 amplification rate in MFS is low, but some cases do show this genetic alteration. Pathologists should be aware of this to avoid misclassification. Further studies are needed to determine the prognostic value of the amplification status.
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY
(2023)
Review
Pathology
Federico Repetto, Deepika Sirohi, Patricia Greipp, Jonathon Mahlow
Summary: TFEB-amplified renal cell carcinoma is characterized by genomic amplification at the 6p21.1 locus and is important to accurately diagnose to determine prognosis and treatment options.
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY
(2023)
Article
Hematology
Jon L. Ocal, Andrew L. Feldman, Patricia T. Greipp, Lisa M. Rimsza
Summary: This case report of a 23-year-old female with PMBL-TH highlights the importance of immunohistochemistry, FISH, and genetic expression analysis in diagnosis, expanding the understanding of DH/TH in B-cell lymphomas.
JOURNAL OF HEMATOPATHOLOGY
(2022)