Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis

Lee, Jerry C.; Logan, Aaron C.

doi:10.3390/cancers15061839

Cited by 12 publications

(2 citation statements)

References 169 publications

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“…It is estimated that around 50% of secondary HLH cases in North America and Europe are due to malignancy, while the other half of the cases are associated with rheumatological diseases or infections [5]. HLH has been reported in association with T-cell lymphoma in multiple instances [6][7][8][9] (see…”

Section: Discussionmentioning

confidence: 99%

A Case of Secondary Hemophagocytic Lymphohistiocytosis in a Patient With T-cell Lymphoma

Jahshan,

Owczarczyk,

Daw

et al. 2024

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition that results from excessive immune activation and inflammation. This condition may be triggered by various factors, including infections, malignancies, or autoimmune diseases. Here, we report the case of a 39-year-old male who developed HLH secondary to T-cell lymphoma and had a history of multiple autoimmune disorders.Our patient presented with shortness of breath and weakness which led to an admission for methicillinresistant Staphylococcus aureus bacteremia. His hospital course deteriorated rapidly due to his worsening condition. He was confirmed to have HLH based on the HLH-2004 criteria with the presence of fever, splenomegaly, hypertriglyceridemia, hypofibrinogenemia, low natural killer cell function, high ferritin, and soluble interleukin 2 receptor levels. Peripheral blood smear and bone marrow biopsy showed atypical lymphocytes consistent with a T-cell lymphoma, but no hemophagocytosis. He was treated with dexamethasone and etoposide. Despite treatment, the patient passed away. This case aims to contribute further to the understanding of secondary HLH in the setting of T-cell lymphoma. It also illuminates how vital early recognition and treatment are in patients with secondary HLH.

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Section: Discussionmentioning

confidence: 99%

A Case of Secondary Hemophagocytic Lymphohistiocytosis in a Patient With T-cell Lymphoma

Jahshan,

Owczarczyk,

Daw

et al. 2024

Cureus

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“…Malignant lymphoma is categorized into low-grade and high-grade lymphoma. It is worth noting that LAHS can manifest as a symptom of high-grade lymphoma rather than low-grade lymphoma ( 3 , 4 ) and is associated with poor prognosis ( 5 ). Although LAHS is usually treated with corticosteroid therapy and cytotoxic chemotherapy for lymphoma ( 6 ), the significance of surgical intervention remains unclear, as no surgical cases have been reported to date.…”

Section: Introductionmentioning

confidence: 99%

Successful treatment of severe splenic lymphoma‑associated hemophagocytic syndrome by splenectomy and subsequent chemotherapy: A case report

Masui,

Shindo,

Inoue

et al. 2024

Oncol Lett

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Hemophagocytic lymphohistiocytosis (HLH) represents a fatal immunopathology derived from excessive inflammatory reactions. In particular, lymphoma-associated hemophagocytic syndrome (LAHS) is associated with a dismal prognosis. The current study presented a challenging case of splenic LAHS. A 71-year-old man presented with fatigue and anorexia. Laboratory test results revealed anemia, thrombocytopenia, lactate dehydrogenase elevation and markedly elevated levels of ferritin (6,210 ng/ml) and soluble interleukin 2 receptor (sIL-2R; 11,328 U/ml). Abdominal computed tomography revealed marked splenomegaly, while fluorodeoxyglucose positron emission tomography revealed increased tracer uptake in the spleen. An elective splenectomy was performed, which led to the diagnosis of B-cell splenic lymphoma with transformation from indolent to aggressive lymphoma. Prior to the splenectomy, thrombocytopenia and hepatic dysfunction with rapidly progressing jaundice appeared, accompanying further elevation of ferritin (25,197 ng/ml) and sIL-2R levels (30,420 U/ml). On postoperative day 5, the patient was transferred to a tertiary care institution and corticosteroid pulse therapy was immediately initiated after establishing the diagnosis of LAHS. Liver dysfunction gradually recovered and subsequent chemotherapy resulted in complete remission with improved performance status. At eight months after the onset, the patient remains alive without any signs of residual lymphoma. Although splenic lymphoma typically manifests with low-grade lymphoma, it can transform into high-grade lymphoma associated with severe complications, such as HLH and multiple organ failure. In this case, splenectomy assisted in not only establishing the diagnosis but also in tumor cytoreduction before commencing chemotherapy. Through interdisciplinary collaboration, the patient was successfully treated by performing a timely splenectomy, followed by steroid pulse therapy and chemotherapy.

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A Novel Engineering Cell Therapy Platform Mimicking the Immune Thrombocytopenia‐Derived Platelets to Inhibit Cytokine Storm in Hemophagocytic Lymphohistiocytosis

Liu,

Du,

Zhou

et al. 2024

Advanced Science

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Hemophagocytic lymphohistiocytosis (HLH) is a common and highly fatal hyperinflammatory syndrome characterized by the aberrant activation of macrophages. To date, there is a lack of targeted therapies for HLH. It is validated that macrophages in HLH efficiently phagocytose anti‐CD41‐platelets (anti‐CD41‐PLTs) from immune thrombocytopenia (ITP) patients in previous research. Hence, the pathological mechanisms of ITP are mimicked and anti‐CD41‐PLTs are utilized to load the macrophage‐toxic drug VP16 to construct macrophage‐targetable engineered platelets anti‐CD41‐PLT‐VP16, which is a novel targeted therapy against HLH. Both in vitro and in vivo studies demonstrate that anti‐CD41‐PLT‐VP16 has excellent targeting and pro‐macrophage apoptotic effects. In HLH model mice, anti‐CD41‐PLT‐VP16 prevents hemophagocytosis and inhibits the cytokine storm. Mechanistic studies reveal that anti‐CD41‐PLT‐VP16 increases the cytotoxicity of VP16, facilitating precise intervention in macrophages. Furthermore, it operates as a strategic “besieger” in diminishing hyperinflammation syndrome, which can indirectly prevent the abnormal activation of T cells and NK cells and reduce the Ab‐dependent cell‐mediated cytotoxicity effect. The first platelet‐based clinical trial is ongoing. The results show that after treatment with anti‐CD41‐PLT‐VP16, HLH patients have a threefold increase in the overall response rate compared to patients receiving conventional chemotherapy. In conclusion, anti‐CD41‐PLT‐VP16 provides a general insight into hyperinflammation syndrome and offers a novel clinical therapeutic strategy for HLH.

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Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis

Cited by 12 publications

References 169 publications

A Case of Secondary Hemophagocytic Lymphohistiocytosis in a Patient With T-cell Lymphoma

A Case of Secondary Hemophagocytic Lymphohistiocytosis in a Patient With T-cell Lymphoma

Successful treatment of severe splenic lymphoma‑associated hemophagocytic syndrome by splenectomy and subsequent chemotherapy: A case report

A Novel Engineering Cell Therapy Platform Mimicking the Immune Thrombocytopenia‐Derived Platelets to Inhibit Cytokine Storm in Hemophagocytic Lymphohistiocytosis

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