Hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma

Mahtat, El Mehdi; Zine, Maryem; Allaoui, Mohamed; Kerbout, Malika; Messaoudi, N.; Doghmi, Kamal; Mikdame, M.

doi:10.1186/s12878-016-0065-5

Cited by 6 publications

(6 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The most common causes of secondary HLH in adults are infections (49%), neoplasms (27%), rheumatoid arthritis (7%), and immunodeficiencies (6%) [ 6 ]. Excessive cytokine secretion by T lymphocytes leads to overactivation of macrophages, causing multiorgan failure.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury

Booth

Osehobo²,

Rodgers-Soriano³

et al. 2018

Case Rep Gastroenterol

View full text Add to dashboard Cite

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon disease that often presents with nonspecific findings. A high index of suspicion is necessary to make a prompt diagnosis and prevent fatal disease. A 45-year-old man presented with fever, hypotension, abdominal pain, nausea, and vomiting. Imaging showed hepatosplenomegaly and laboratory tests revealed pancytopenia, increased ferritin, and a cholestatic pattern of injury with elevated alkaline phosphatase and total bilirubin. Due to a history of Crohn disease, systemic lupus erythematous, and rheumatoid arthritis, the patient was on immunosuppressants, including infliximab. After multiple negative cultures, persistent fever, and days of empiric broad spectrum antibiotics, our differential shifted to fever of unknown origin. A liver wedge biopsy revealed areas of sinusoidal dilatation with enlarged, activated macrophages containing erythrocytes and intracytoplasmic iron, consistent with hemophagocytosis due to HLH. The portal tracts showed mixed lymphoplasmacytic inflammation, a prominent bile ductular reaction, periportal fibrosis, and scattered large cells with occasional binucleation and prominent nucleoli. These cells stained positive for Epstein-Barr virus encoding region in situ hybridization, PAX5, CD15, and CD30, and hepatic involvement by classic Hodgkin lymphoma was diagnosed and determined to be the cause of the HLH and cholestatic pattern of injury. Simultaneously, a bone marrow biopsy showed diffuse involvement by Hodgkin lymphoma with a similar staining pattern. Aggressive treatment failed and the patient succumbed to multiorgan failure. HLH is a rare, potentially fatal disease, with nonspecific signs and symptoms, and should be considered in any patient presenting with fever and pancytopenia, especially if they are immune compromised.

show abstract

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury

Booth

Osehobo²,

Rodgers-Soriano³

et al. 2018

Case Rep Gastroenterol

View full text Add to dashboard Cite

show abstract

“…To date, only five cases of HLH occurring in the setting of THRLBCL have been described in the English literature [ 7 – 11 ]. Of the prior documented cases [ Table 1 ], two achieved complete remission (CR) with R-CHOP, with one of the patients relapsing ten months later [ 6 , 8 ]. A third patient achieved CR with DA-R-EPOCH; and a fourth patient achieved CR with high-dose chemotherapy followed by autologous stem cell transplantation [ 9 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease

Ibrahim

Garcia

Saqib

et al. 2017

Case Reports in Oncological Medicine

View full text Add to dashboard Cite

T cell histiocyte rich large B cell lymphoma (THRLBCL) is a rare subtype of non-Hodgkin's lymphoma characterized by malignant B cells with reactive T lymphocytes. The pathophysiology is thought to involve cytokine-mediated evasion of T cell immune response by malignant B cells. It usually presents at an advanced stage with extranodal involvement. An extremely unusual manifestation of the disease is hemophagocytic lymphohistiocytosis (HLH) which is a hyperinflammatory disorder. We present a case of a 43-year-old male who presented with recurrent fever and recent radiologic imaging showing splenomegaly and right inguinal lymphadenopathy. On presentation, he had a fever of 105°F. Laboratory work-up was consistent with pancytopenia, elevated lactate dehydrogenase, elevated D-dimer, and a ferritin of 24,247 ng/mL. The patient was started on steroid therapy. An excisional biopsy of the right inguinal lymph node was consistent with a diagnosis of THRLBCL and the patient subsequently received six cycles of chemotherapy with R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) after which a PET-CT scan showed no evidence of biologically active disease and ferritin was down to 822 ng/mL. We discuss the clinical manifestations and diagnostic and therapeutic considerations of this rare disease along with a review of reported cases in the literature.

show abstract

“…Our patient had 6 of the 8 clinical criteria: fever, splenomegaly, hypertriglyceridemia, hyperferritinemia, increased soluble CD25 levels, and hemophagocytosis in bone marrow. The most common causes of secondary HLH in adults are infections (49%), neoplasms (27%), rheumatoid arthritis (7%), and immunodeficiencies (6%) [6] . On the first admission to Blood Transfusion Hematology Hospital, with negative results on autoimmune screening tests, full body CT Scan and digestive system endoscopy, the patient was diagnosed with HLH probably secondary to Epstein-Barr virus (EBV) infection because of positive result on EBV detection by PCR.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma

Cited by 6 publications

References 13 publications

Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury

Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury

T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease

Hemophagocytic lymphohistiocytosis secondary to diffuse large B-cell lymphoma presenting with recurrent multi-territory infarcts: A case report

Contact Info

Product

Resources

About