An unusual indication for splenectomy in hairy cell leukaemia: a report of three cases with persistent splenomegaly after chemoimmunotherapy

Sarid, Nadav; Ahmad, Humayun N.; Wotherspoon, Andrew; Dearden, Claire; Else, Monica; Catovsky, Daniel

doi:10.1111/bjh.13767

Cited by 7 publications

(2 citation statements)

References 10 publications

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“…Even with no residual HCL, splenomegaly can persist due to benign elements and the associated hypersplenism can cause thrombocytopenia. Splenectomy should be avoided for these patients [ 100 ]. Flow cytometry of the blood and assays of soluble forms of CD25 or CD22 [ 101 ] may be useful assessing residual HCL burden with splenomegaly.…”

Section: How Response To Treatment Is Determined In Hclmentioning

confidence: 99%

Diagnosis and treatment of hairy cell leukemia as the COVID-19 pandemic continues

Kreitman

Arons

2022

Blood Reviews

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Hairy cell leukemia (HCL) is an indolent B-cell malignancy, usually driven by the BRAF V600E mutation. For 30 years, untreated and relapsed HCL was successfully treated with purine analogs, but minimal residual disease (MRD) remained in most patients, eventually causing relapse. Repeated purine analogs achieve decreasing efficacy and increasing toxicity, particularly to normal T-cells. MRD-free complete remissions (CRs) are more common using rituximab with purine analogs in both 1 st -line and relapsed settings. BRAF inhibitors and Ibrutinib can achieve remission, but due to persistence of MRD, must be used chronically to prevent relapse. BRAF inhibition combined with Rituximab can achieve high MRD-free CR rates. Anti-CD22 recombinant immunotoxin moxetumomab pasudotox is FDA-approved in the relapsed setting and is unique in achieving high MRD-free CR rates as a single-agent. Avoiding chemotherapy and rituximab may be important in ensuring both recovery from COVID-19 and successful COVID-19 vaccination, an area of continued investigation.

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Section: How Response To Treatment Is Determined In Hclmentioning

confidence: 99%

Diagnosis and treatment of hairy cell leukemia as the COVID-19 pandemic continues

Kreitman

Arons

2022

Blood Reviews

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“…A ≥50% reduction in products of lymph node perpendicular diameters or reduction to a size consistent with CR by CT or MRI is considered consistent with PR. It is notable that residual splenomegaly and hypersplenism causing thrombocytopenia may remain in the absence of residual disease, and splenectomy may not be indicated in these cases [ 69 ]. Blood flow cytometry and serum soluble CD25 or CD22 [ 70 ] may be helpful in ruling out residual splenic involvement.…”

Section: Criteria For Response In Hclmentioning

confidence: 99%

Development of Recombinant Immunotoxins for Hairy Cell Leukemia

Kreitman

Pastan

2020

Biomolecules

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Hairy cell leukemia (HCL) is an indolent B-cell malignancy with excellent initial response to purine analogs pentostatin or cladribine, but patients are rarely, if ever, cured. Younger patients will usually need repeat chemotherapy which has declining benefits and increasing toxicities with each course. Targeted therapies directed to the BRAF V600E mutation and Bruton’s tyrosine kinase may be helpful, but rarely eradicate the minimal residual disease (MRD) which will eventually lead to relapse. Moxetumomab pasudotox (Moxe) is an anti-CD22 recombinant immunotoxin, which binds to CD22 on HCL cells and leads to apoptotic cell death after internalization and trafficking of the toxin to the cytosol. Phase I testing achieved a complete remission (CR) rate of 57% in relapsed/refractory HCL. Most CRs were without MRD and eradication of MRD correlated with prolonged CR duration. Patients were often MRD-free after five years. Important mild-moderate toxicities included capillary leak and hemolytic uremic syndromes which could be prevented and managed conservatively. A phase 3 trial met its endpoint of durable CR with acceptable toxicity, leading to FDA approval of Moxe for relapsed/refractory HCL, under the name Lumoxiti. Moxe combined with rituximab is currently being evaluated in relapsed/refractory HCL to improve the rate of MRD-free CR.

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