scholarly journals Hemophagocytic Lymphohistiocytosis as the Initial Presentation of Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Rare Case Responding to Cyclosporine A and Steroids

2020 ◽  
Vol 8 ◽  
pp. 232470962098153
Author(s):  
Caitlin Sullivan ◽  
Arya Loghmani ◽  
Katharine Thomas ◽  
Rachna Jetly-Shridhar ◽  
Rajasree Pia Chowdry
Keyword(s):  
T Cell ◽  
Cyclosporine A ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Cell Lymphoma ◽  
Skin Lesions ◽  
T Cell Lymphoma ◽  
Cytotoxic T Cell ◽  
Life Threatening ◽  
History Of ◽  
Rare Malignancy

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare peripheral cytotoxic T-cell lymphoma, clinically resembling panniculitis. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of immune overactivation, triggered by underlying conditions. SPTCL presenting with HLH may represent a severe and rapidly progressive disease course. Currently, there is no standardized approach to treatment of HLH secondary to underlying SPTCL. A 34-year-old Asian male presented with a several months history of high fevers, weight loss, and nonpruritic purple discoloration of the skin. He had a skin biopsy showing atypical lymphohistiocytic panniculitis with dermal mucinosis and erythrophagocytosis consistent with SPTCL. The patient was initiated on treatment with dexamethasone and cyclosporine A. Almost immediate improvement of his skin lesions was noted and laboratory abnormalities trended toward baseline within 2 weeks. He noted complete symptom resolution after 3 months on therapy. SPTCL may be treated effectively with cyclosporine A and steroids to achieve rapid clinical and symptom management of this rare malignancy.

Hemophagocytic Lymphohistiocytosis in Adults: Updates in Diagnosis and Treatment

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5037-5037
Author(s):  
Roman Leonid Kleynberg ◽  
Gary J. Schiller
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Cyclosporine A ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Research Funding ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
T Cell Lymphoma ◽  
White Blood Count ◽  
History Of

Abstract Abstract 5037 Hemophagocytic lymphohistiocytosis (HLH) is rare potentially life-threatening disorder that must be recognized and treated early to improve clinical response rates and outcomes. We describe a 36 year-old male with a longstanding history of ulcerative colitis who presented with a one-week history of lymphadenopathy, splenomegaly, fevers, and chills. Workup at an outside institution had been intensive - he had a long complicated hospital course that included hypoxemic respiratory failure requiring intubation and mechanical ventilation, acute kidney injury, hyperbilirubinemia, pancytopenia, coagulopathy, hypofibrinogenemia, CMV viremia, and Clostridium difficile colitis for which he was treated. At our facility, the patient presented with a white blood count of 0.19 109/L (N: 5%, L: 95%, M: 0%, E: 0%, B: 0%), hemoglobin 9.0 mg/L, platelets 54 x109/L, ferritin 538 ng/dL, and triglycerides of 280 mg/dL. A bone marrow biopsy revealed CMV positivity by immunohistochemistry and marked hemophagocytic histiocytosis. The patient was treated with the HLH-2004 chemo-immunotherapy protocol with dexamethasone, etoposide (VP-16), and cyclosporine A. He responded well and is now eight months status-post initial treatment. HLH in adults is a disorder that little is known about. Acquired forms of HLH can occur in otherwise healthy adults, whereas in children, HLH often presents in its inherited (familial) form. In adults it is often associated with infections (i.e. Epstein-Barr virus, herpes simplex, cytomegalovirus), rheumatologic diseases (i.e. rheumatoid arthritis, systemic lupus erythematosus, adult onset systemic Still's disease), malignancy (i.e. natural killer cell leukemia, peripheral T-cell lymphoma, EBV in T-cell lymphoma, B-cell lymphoma, and a variety of other lymphomas). HLH in adults is characterized by cytokine dysfunction and results in the systemic accumulation of activated T-lymphocytes and activated histiocytes (macrophages). Natural killer (NK)-cell and cytotoxic T cell activity in HLH patients is commonly depressed. The standard chemo-immunotherapy protocol as recommended by the Histiocyte Society (HLH-2004) includes therapy with dexamethasone, etoposide (VP-16), cyclosporine A upfront and, in selected patients, cortiocosteriods and intrathecal methotrexate. Thus, early recognition and treatment of this disorder is essential to decrease associated morbidity/mortality. Hemophagocytic lymphohistiocytosis in adults is a diagnosis that must be in the differential whenever a patient presents with fever, cytopenia, hepatosplenomegaly, hypertriglyceridemia and/or hypofibrinogenemia. Disclosures: Schiller: BMS: Research Funding; Celgene: Research Funding; Ambit: Research Funding; Novartis: Research Funding; Sunesis: Research Funding.

Malodorous Skin Lesions in a Diabetic Patient: Infectious Versus Neoplastic

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S113-S114
Author(s):  
Ismail Elbaz Younes ◽  
Julia Rewerska ◽  
Victoria Alagiozian-Angelova
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Clinical Information ◽  
Skin Lesions ◽  
T Cell Lymphoma ◽  
Cytotoxic T Cell ◽  
Lymphoid Tissues ◽  
Rare Entity ◽  
Lymphomatoid Papulosis ◽  
Type D

Abstract Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma is a rare entity accounting for <1% of all cutaneous T-cell lymphomas. Almost all patients present with generalized skin lesions. This type of lymphoma has an extremely aggressive course with a median survival of 12 months. It tends to spread to other visceral sites, but lymph nodes are usually spared. We describe a case of a 59-year-old male with multiple necrotic malodours ulcers for several months. The first lesion was on his left thigh, followed by another lesion on his right chest and right eyelid. Medical history revealed newly diagnosed diabetes mellitus. The patient received antibiotics, presumptively for infectious etiology of the skin lesion, with no improvement. The right thigh lesion was excised and histomorphologic examination revealed a deep dermal proliferation of large-sized pleomorphic cells with marked pagetoid epidermotropism and skin ulceration. The adnexal skin structures were invaded by the lesion. The lesional cells were immunoreactive for CD3, CD7, CD8, and granzyme B; they were negative for CD4, CD5, CD56, and CD30. The immunophenotype confirms the entity that we have at hand in addition to the similar clinical picture that the patient presented with. This disease usually shows clonal TR gene rearrangements; nonetheless, no specific mutational aberration has been described. Our patient received chemotherapy; however, new lesions continued to erupt and he opted to proceed with palliative care. Clinical information is needed to give this diagnosis as it may look identical to a variant of lymphomatoid papulosis (type D), CD8-positive cutaneous T-cell lymphoma. We present this case due to the importance of clinical pathologic coloration to prevent misdiagnosis with mimickers as the ones pointed out earlier, and it is a provisional rare entity in the 2018 WHO classification of Tumors of Haematopoietic and Lymphoid Tissues.

scholarly journals Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting as a Local Inflammation of a Thigh in an 8-Month-Old Child

2017 ◽  
Vol 05 (01) ◽  
pp. e68-e70 ◽  
Author(s):  
Kaja Gizewska-Kacprzak ◽  
Katarzyna Karpinska-Kaczmarczyk ◽  
Tomasz Ociepa
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Histopathological Examination ◽  
Skin Inflammation ◽  
T Cell Lymphoma ◽  
Pediatric Care ◽  
Cytotoxic T Cell ◽  
Local Inflammation ◽  
Surgical Biopsy ◽  
History Of

AbstractDuring infancy, skin inflammation is usually treated in basic pediatric care. In this study, we present a case of an 8-month-old girl with a 2-month history of an inflammation of the thigh treated locally by ointments and oral antibiotics in basic and dermatological care. The patient had a history of fever, sweating, and failure to thrive. The lactate dehydrogenase was elevated up to 869 U/L with low C-reactive protein (1.04 mg/L). Magnetic resonance imaging of the thigh reassured the diagnosis of local inflammation. Intravenous antibiotic caused mild local improvement, but the episodes of high fever sustained. The patient was transferred to our pediatric surgery department for treatment and surgical biopsy of the lesion. Histopathological examination confirmed a subcutaneous panniculitis-like T-cell lymphoma, which is a rare cytotoxic T-cell lymphoma representing less than 1% of non-Hodgkin lymphomas, uncommon in children. The patient was introduced to a chemotherapy protocol EURO-LB 2002 with good response. In a skin lesion that is associated with systemic symptoms and responding untypically to antibiotic treatment malignancy should be considered and biopsy not be postponed.

scholarly journals A case of canine renal lymphoma of granular lymphocytes with severe polycythemia

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Sara Kotb ◽  
Carolina Allende ◽  
T. William O’Neill ◽  
Krista Bruckner ◽  
Helio DeMorais ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Left Kidney ◽  
Abdominal Ultrasound ◽  
The Body ◽  
T Cell Lymphoma ◽  
Cytotoxic T Cell ◽  
Renal Lymphoma ◽  
History Of ◽  
Granular Lymphocytes

Abstract Background Renal lymphoma in dogs is rare and has a poor prognosis. Granular lymphocyte morphology is rarely reported in canine renal lymphoma. Mild to moderate polycythemia is reported in a number of canine renal lymphoma cases. Case presentation A 10-year-old Labrador retriever presented to a university veterinary teaching hospital after a 1-month history of polyuria, polydipsia, and pollakiuria and a 2-week history of abdominal distention, lethargy, and increased respiratory effort. Abdominal ultrasound showed a wedge-shaped to rounded, heterogeneously hypoechoic mass lesion in the left kidney. Cytologic analysis of a percutaneous aspirate of the mass was consistent with lymphoma of granular lymphocytes. Severe polycythemia (hematocrit 0.871) was noted on a complete blood cell count. Clonality analysis identified a clonally rearranged T-cell receptor (TCR) gene and immunohistochemical staining was CD3+, CD79a- and CD11d+, supporting cytotoxic T-cell lymphoma. Conclusions To our knowledge, this is the first report of renal cytotoxic T-cell lymphoma with severe polycythemia in a dog. Severe polycythemia and renal cytotoxic T-cell lymphoma are both rare in dogs; this report adds to the body of knowledge on these conditions.

scholarly journals Primary NK/T cell lymphoma nasal type of the stomach with skin involvement: a case report

Rare Tumors ◽  
2009 ◽  
Vol 1 (2) ◽  
pp. 173-175
Author(s):  
Sebastian Kobold ◽  
Hartmut Merz ◽  
Markus Tiemann ◽  
Carolina Mahuad ◽  
Carsten Bokemeyer ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Skin Lesions ◽  
Primary Site ◽  
T Cell Lymphoma ◽  
Skin Involvement ◽  
Unique Case ◽  
Nasal Type ◽  
History Of ◽  
Nk T Cell

Since nasal NK/T cell lymphoma and NK/T cell lymphoma nasal type are rare diseases, gastric involvement has seldom been seen. We report a unique case of a patient with a primary NK/T cell lymphoma nasal type of the stomach with skin involvement. The patient had no history of malignant diseases and was diagnosed with hematemesis and intense bleeding from his gastric primary site. Shortly after this event, exanthemic skin lesions appeared with concordant histology to the primary site. Despite chemotherapy, the patient died one month after the first symptomatic appearance of disease.

Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients

Blood ◽  
2003 ◽  
Vol 102 (6) ◽  
pp. 2213-2219 ◽  
Author(s):  
Marcel W. Bekkenk ◽  
Maarten H. Vermeer ◽  
Patty M. Jansen ◽  
Ariënne M. W. van Marion ◽  
Marijke R. Canninga-van Dijk ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Size ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Skin Lesions ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
Nor Phenotype ◽  
Peripheral T Cell Lymphomas

Abstract In the present study the clinicopathologic and immunophenotypic features of 82 patients with a CD30– peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out whether subdivision of these lymphomas on the basis of cell size, phenotype, or presentation with only skin lesions is clinically relevant. The study group included 46 primary cutaneous CD30– large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphomas with both skin and extracutaneous disease at the time of diagnosis. Patients with primary cutaneous small- or medium-sized T-cell lymphomas had a significantly better prognosis (5-year-overall survival, 45%) than patients with primary cutaneous CD30– large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this group with primary cutaneous small- or medium-sized T-cell lymphomas was particularly found in patients presenting with localized skin lesions expressing a CD3+CD4+CD8– phenotype. In the primary cutaneous T-cell lymphoma (CTCL) group and in the concurrent group, neither extent of skin lesions nor phenotype had any effect on survival. Our results indicate that peripheral T-cell lymphomas, unspecified, presenting in the skin have an unfavorable prognosis, irrespective of the presence or absence of extracutaneous disease at the time of diagnosis, cell size, and expression of a CD4+ or CD8+ phenotype. The only exception was a group of primary cutaneous small- or medium-sized pleomorphic CTCLs with a CD3+CD4+CD8– phenotype and presenting with localized skin lesions.

Sign in / Sign up

Export Citation Format

Share Document