Chemotherapy and radiation therapy for the treatment of other single-system disease and multisystem disease

A single-center, retrospective review of 58 adult patients with LCH reported on the efficacy and toxicities of treatment with vinblastine/prednisone, cladribine, and cytarabine.[51][Level of evidence C3]

A single-center, retrospective review of 58 adult patients with LCH reported on the efficacy and toxicities of treatment with vinblastine/prednisone, cladribine, and cytarabine.[51][Level of evidence C3]

Patients treated with vinblastine/prednisone had the worst outcome, with 84% of patients not responding within 6 weeks or relapsing within a year.

A single-center, retrospective review of 58 adult patients with LCH reported on the efficacy and toxicities of treatment with vinblastine/prednisone, cladribine, and cytarabine.[51][Level of evidence C3]

The no-response/relapse rate was 59% for patients treated with cladribine and 21% for patients treated with cytarabine.

A single-center, retrospective review of 58 adult patients with LCH reported on the efficacy and toxicities of treatment with vinblastine/prednisone, cladribine, and cytarabine.[51][Level of evidence C3]

Grade 3 or 4 neurological toxic effects occurred in 75% of patients treated with vinblastine.

A single-center, retrospective review of 58 adult patients with LCH reported on the efficacy and toxicities of treatment with vinblastine/prednisone, cladribine, and cytarabine.[51][Level of evidence C3]

Grade 3 or 4 neutropenia occurred in 37% of patients treated with cladribine and in 20% of patients who received cytarabine.

One report evaluated adult patients who were treated with either vindesine and prednisone or cyclophosphamide, etoposide, vindesine, and prednisone.[71][Level of evidence C2]

One report evaluated adult patients who were treated with either vindesine and prednisone or cyclophosphamide, etoposide, vindesine, and prednisone.[71][Level of evidence C2]

More than 70% of patients relapsed with either regimen.

Etoposide has been used with some success in adult patients with single-system and multisystem LCH.

Etoposide has been used with some success in adult patients with single-system and multisystem LCH.

Minimal toxicity was reported with the use of prolonged oral etoposide in adults with skin LCH, while 3-day courses of intravenous etoposide (100 mg/m2/day) induced complete remission in a small number of patients with resistant single-system and multisystem disease.[72][Level of evidence C3]

Etoposide has been used with some success in adult patients with single-system and multisystem LCH.

Another study at the same center found that azathioprine was the most successful drug for localized disease in adults, with the addition of etoposide for refractory and multisystem disease.[73][Level of evidence C3]

Cladribine is effective for adults with skin, bone, lymph node, and probably pulmonary and CNS disease.[74,75][Level of evidence C3]; [52]

Cladribine is effective for adults with skin, bone, lymph node, and probably pulmonary and CNS disease.[74,75][Level of evidence C3]; [52]

In a retrospective multicenter study, 23 patients who had at least one previous therapy were treated with cladribine, using various dosing and treatment schedules.[76][Level of evidence C2] The overall response rate was 91%, and the complete response rate was 50%. A literature review identified an additional 48 patients who were treated with cladribine. The pooled analysis confirmed these results.

Cladribine is effective for adults with skin, bone, lymph node, and probably pulmonary and CNS disease.[74,75][Level of evidence C3]; [52]

A study reported on 38 patients with newly diagnosed or relapsed/refractory LCH treated with one to nine cycles of cladribine.[1][Level of evidence C2] The overall response rate was 79%. The complete response rate was 26%, and the partial response rate was 53%.[52]

An adult lymphoma treatment regimen of methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin (MACOP-B) was used in 11 patients.[77]

An adult lymphoma treatment regimen of methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin (MACOP-B) was used in 11 patients.[77]

The overall response rate was 100%, and the progression-free survival rate was 64%.

Methotrexate and cytarabine were given to 83 patients with newly diagnosed lung (68%), liver (28%), spleen (13%), or nonpituitary (4%) LCH.[78]

Methotrexate and cytarabine were given to 83 patients with newly diagnosed lung (68%), liver (28%), spleen (13%), or nonpituitary (4%) LCH.[78]

The objective response rate was 88%, and one-third of the patients progressed after 3 years.

Methotrexate and cytarabine were given to 83 patients with newly diagnosed lung (68%), liver (28%), spleen (13%), or nonpituitary (4%) LCH.[78]

There was a high rate of grades 3 to 4 neutropenia, with nearly one-half of patients developing fevers.

Methotrexate and cytarabine were given to 83 patients with newly diagnosed lung (68%), liver (28%), spleen (13%), or nonpituitary (4%) LCH.[78]

One-third of patients had grades 3 to 4 thrombocytopenia.

In a study of 61 adult patients with LCH who were treated with subcutaneous cytarabine, the following results were reported:[79]

In a study of 61 adult patients with LCH who were treated with subcutaneous cytarabine, the following results were reported:[79]

The estimated 3-year event-free survival (EFS) rate was 58.5%.

In a study of 61 adult patients with LCH who were treated with subcutaneous cytarabine, the following results were reported:[79]

Poor prognostic factors for EFS included three or more involved organs and baseline lung involvement.

In a study of 61 adult patients with LCH who were treated with subcutaneous cytarabine, the following results were reported:[79]

Grades 3 to 4 neutropenia occurred in 27.9% of patients.

In a study of 61 adult patients with LCH who were treated with subcutaneous cytarabine, the following results were reported:[79]

Of the 61 patients, 47 completed 12 months of treatment, and 14 left the study (6 had a poor response to therapy and 8 decided to withdraw).