scholarly journals ALK-Negative Anaplastic Large Cell Lymphoma Presenting as Disseminated Intravascular Coagulation and Hemophagocytic Lymphohistiocytosis: A Potentially Fatal Presentation

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Uroosa Ibrahim ◽  
Amina Saqib ◽  
Maryam Rehan ◽  
Jean Paul Atallah

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that can be familial in etiology or a result of infections, malignancy, and autoimmune or inflammatory disorders. Disseminated intravascular coagulation (DIC) is common in patients admitted to intensive care units and can confound and delay the diagnosis of HLH. We present a case of a 69-year-old female who presented with dyspnea and malaise. Her condition declined rapidly with laboratory parameters consistent with DIC. In addition, she had a ferritin of 32,522 ng/mL, low haptoglobin, and elevated LDH, and bone marrow biopsy showed hemophagocytic lymphohistiocytes. She was started on HLH-directed therapy, and later, a diagnosis of ALK-negative anaplastic large cell lymphoma was made on an excisional inguinal lymph node biopsy specimen. Our case emphasizes the importance of prompt recognition, diagnosis, and treatment of HLH while workup for a primary disorder is still being pursued.

2014 ◽  
Vol 2014 ◽  
pp. 1-7
Author(s):  
Aneesh Basheer ◽  
Somanath Padhi ◽  
Ramesh Nagarajan ◽  
Vinoth Boopathy ◽  
Sudhagar Mookkappan ◽  
...  

Hemophagocytic lymphohistiocytosis (HLH) has a well known association with lymphomas, especially of T cell origin. Prognosis of lymphoma associated HLH is very poor, especially in T cell lymphomas; and, therefore, early diagnosis might alter the outcome. Though association of HLH with systemic anaplastic large cell lymphoma (ALCL) is known, its occurrence in primary cutaneous ALCL (C-ALCL) is distinctly rare. We aim to describe a case of C-ALCL (anaplastic lymphoma kinase (ALK)−) in an elderly male who succumbed to the complication of associated HLH, which was possibly triggered by coexistent virus infection. We briefly present the literatures on lymphoma associated HLH and discuss the histopathological differentials of cutaneous CD30+ lymphoproliferative disorders. We do suggest that HLH may pose diagnostic challenges in the evaluation of an underlying lymphoma and hence warrants proper evaluation for the underlying etiologies and/or triggering factors.


2007 ◽  
Vol 49 (7) ◽  
pp. 1056-1056 ◽  
Author(s):  
Zdenka Krenova ◽  
Jaroslav Sterba ◽  
Jan Blatny ◽  
Leos Kren ◽  
Jaroslav Slany

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Barr D ◽  
◽  
Maslov D ◽  
Goel R ◽  
Field H ◽  
...  

Anaplastic Large Cell Lymphoma (ALCL) is a rare aggressive neoplasm. Rapid and progressive lymphadenopathy is common. Due to its aggressive nature, two-thirds of initial presentations are in stage III or IV. Multifocal disease that is primarily cutaneous is rare and extracutaneous spread of the cancer occurs in up to 13 percent of cases. In this case, a patient with systemic ALCL went undiagnosed for two months across three hospitalizations. A 33-year old male presented with multiple cutaneous lesions that had erupted bilaterally on his lower extremities and a fever which was unresponsive to over the counter medication. The patient required three hospitalizations and extensive work up before a diagnosis of Anaplastic Lymphoma Kinase (ALK) -negative CD30+ T-cell lymphoma was made by skin and lymph node biopsy. Diagnosis was delayed due to this patient’s uncommon presentation, the broad list of differential diagnoses, inaccuracies in biopsy, and communication delays from multiple hospital visits. This patient had aggressive ALCL and passed away during the first week of chemotherapy treatment. When there is a concern for malignancy, properly performed biopsies are important to collect from appropriate sites with non-necrotic tissue. Additionally, this case demonstrates the consequences of inadequate communication during handoff between transfer centers. It is important to have a broad differential diagnosis as well as prompt investigation and constant communication between all providers involved when a patient presents with abnormal and aggressively progressing symptoms.


2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Jemima Sharp ◽  
Barry Pizer ◽  
George Kokai ◽  
Marcus K. H. Auth

Screening for coeliac disease is recommended for children from certain risk groups, with implications for diagnostic procedures and dietetic management. The risk of a malignant complication in untreated coeliac disease is not considered high in children. We present the case of a girl with type I diabetes who developed weight loss, fatigue, and inguinal lymphadenopathy. Four years before, when she was asymptomatic, a screening coeliac tTG test was positive, but gluten was not eliminated from her diet. Based on clinical examination, a duodenal biopsy, and an inguinal lymph node biopsy were performed, which confirmed both coeliac disease and an anaplastic large-cell lymphoma. HLA-typing demonstrated that she was homozygous for HLA-DQ8, which is associated with higher risk for celiac disease, more severe gluten sensitivity, and diabetes susceptibility. She responded well to chemotherapy and has been in remission for over 4 years. She remains on a gluten-free diet. This is the first case reporting the association of coeliac disease, type I diabetes, and anaplastic large-cell lymphoma in childhood. The case highlights the malignancy risk in a genetically predisposed individual, and the possible role of a perpetuated immunologic response by prolonged gluten exposure.


2015 ◽  
Vol 90 (8) ◽  
pp. 746-747 ◽  
Author(s):  
Mili Shah ◽  
Leena Karnik ◽  
Elizabet Nadal-Melsió ◽  
Alistair Reid ◽  
Radia Ahmad ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document