Clinicopathological Characteristics of Primary Pulmonary Hodgkin Lymphoma (PPHL): Two Institutional Experiences with Comprehensive Literature Review of 115 PPHL Cases

Abstract

Primary pulmonary Hodgkin lymphoma (PPHL) is an extremely rare condition. Its clinicopathological characteristics remain unclear because of the limited number of patients with PPHL. The aim of this study was to comprehensively analyze the clinicopathological characteristics of PPHL. We reviewed the electronic medical records and pathology slides of our 10 PPHL patients. The female-to-male ratio was 6:4, and the mean age was 41 years. Although three patients had no symptoms, seven had localized or generalized symptoms, including cough, sputum, chest discomfort/pain, and weight loss. Some cases had not been diagnosed as PPHL in the initial needle biopsy. Four patients underwent surgical resection. With chemotherapy, eight patients achieved complete remission. We also conducted a thorough literature review on 105 previously reported PPHL cases. Among a total of 115 PPHL cases, the most common subtype was nodular sclerosis (37.4%). More than half of the cases (55%) were clinically suspected as infectious pneumonia. Of 61 patients whose biopsies were available, 27 (44.3%) were diagnosed correctly as Hodgkin lymphoma, whereas the misdiagnoses included tuberculosis, Langerhans cell histiocytosis, solitary fibrous tumor, and adenocarcinoma. We demonstrated that PPHL represents a diagnostic challenge on small biopsies. Recognizing that this rare tumor can mimic infectious and inflammatory diseases as well as malignancies is important because the accurate diagnosis of PPHL is essential for adequate clinical management.

Programmed Death 1 (PD-1) Expression in Relapsing and Remitting Hodgkin Lymphoma as Prognostic Factor

“…Also, the most common type of HL in this study was Nodular Sclerosis, which is consistent with previous finding (Jung et al, 2022). In examining the relationship between HL type and other variables, two types of Lymphocyte-Rich and Lymphocyte-Depleted were not included due to the very small sample size.…”

“…The mean age of total patients in the study was 34.03±15.78 years, ranging from 8 years to 76 years. This age was lower than previous studies (Jalili et al, 2022;Jung et al, 2022), but another study showed a bimodal age curve (a mode at 15 ~ 35 years of age and the second mode in elderly) (Nakatsuka and Aozasa, 2006). These differences may note the various age specific relation for the HL however a in Iran (Monabati et al, 2020) and China (Jalili et al, 2022) reported a rather similar age.…”

“…By the type of HL, the most common type reported nodular sclerosis type which is estimated 62.3% of all cases. Also, the mean age ± SD of HL cases calculated 46 ± 6.2 y in which it seems to be more in male (73%) than female (27%) (Jung et al, 2022;Vassilakopoulos et al, 2020). Cytotoxic therapy is the main strategy of treatment for majority of HL cases (80-90%) however 15-20% of them became resistant and disease relapse could be seen in these patients (Weniger and Küppers, 2021).…”

Primary pulmonary Hodgkin’s lymphoma mimicking granulomatosis with polyangiitis – a case report of diagnostic and therapeutic dilemmas

“…However, this study included patients who underwent various treatments, including radiation, surgery, or chemotherapy, with the latter primarily being the MOPP regimen (mechlorethamine, vincristine, procarbazine, prednisone), which is currently not recommended. Conversely, recent analyses and individual case reports on ABVD administration are optimistic, demonstrating mostly CR in patients [ 17 , 18 ]. Given these circumstances, the prognosis of PPHL and the outcomes of novel chemotherapy regimens remain uncertain, necessitating further studies involving a larger patient cohort.…”

Hodgkin Lymphoma Mimicking Lung Carcinoma

“…Pulmonary involvement of secondary HL is not uncommon and is reported to occur in 15–40% of patients. 1 , 2 …”