Table 2

Diagnostic workup of CD30+ LPD

Histologic features compatible with LYP or PCALCL 
    History 
        Wax and waning of lesions (ie, spontaneous regression of each lesion within weeks to months) with new ones developing 
        Previous lymphoid neoplasms, particularly Hodgkin lymphoma, nodal anaplastic large cell lymphoma, and MF 
        Immunosuppression (HIV, organ transplantation, or other conditions associated with immunosuppressive therapy, immunosuppression-related CD30+ LPDs) 
        B symptoms (fever, night sweats, weight loss) 
    Physical examination 
        Size and number of lesions 
        Presence of patches and/or plaques indicates possibility of associated MF. 
        It is necessary to differentiate MF with transformation (CD30 may be expressed by large tumor cells in transformed MF) from CD30+ LPD. 
        Enlarged lymph nodes (see point F) 
        Hepatic or splenic enlargement 
    Laboratory investigations 
        Complete blood cell count and differential 
        Blood chemistries, including LDH 
        Serology for HTLV-1/2 (only in areas with endemic HTLV infection) to identify adult T-cell lymphoma/leukemia, in which expression of CD30 by tumor cells can occur 
    Radiologic examinations 
        LYP: Radiologic examinations (chest x-ray, ultrasound abdomen and pelvis, or CT scan) are considered as optional examinations in patients with typical LYP and absence of palpable enlarged lymph nodes, absence of hepatosplenomegaly, normal laboratory tests, and absence of B symptoms. 
        PCALCL: Contrast-enhanced CT scan with or without positron emission tomography (chest, abdomen, pelvis) or whole-body integrated positron emission tomography/CT. 
    Bone marrow aspirate or biopsy 
        LYP: Not performed in patients with typical LYP 
        PCALCL: Optional in patients with solitary PCALCL or patients with PCALCL without extracutaneous involvement in radiologic examinations (D)19  
        Lymph node biopsy: If enlarged lymph nodes (defined as > 1.5 cm in greatest transverse [long axis] diameter) are palpable or enlarged lymph nodes are detected on radiologic examination. 
Histologic features compatible with LYP or PCALCL 
    History 
        Wax and waning of lesions (ie, spontaneous regression of each lesion within weeks to months) with new ones developing 
        Previous lymphoid neoplasms, particularly Hodgkin lymphoma, nodal anaplastic large cell lymphoma, and MF 
        Immunosuppression (HIV, organ transplantation, or other conditions associated with immunosuppressive therapy, immunosuppression-related CD30+ LPDs) 
        B symptoms (fever, night sweats, weight loss) 
    Physical examination 
        Size and number of lesions 
        Presence of patches and/or plaques indicates possibility of associated MF. 
        It is necessary to differentiate MF with transformation (CD30 may be expressed by large tumor cells in transformed MF) from CD30+ LPD. 
        Enlarged lymph nodes (see point F) 
        Hepatic or splenic enlargement 
    Laboratory investigations 
        Complete blood cell count and differential 
        Blood chemistries, including LDH 
        Serology for HTLV-1/2 (only in areas with endemic HTLV infection) to identify adult T-cell lymphoma/leukemia, in which expression of CD30 by tumor cells can occur 
    Radiologic examinations 
        LYP: Radiologic examinations (chest x-ray, ultrasound abdomen and pelvis, or CT scan) are considered as optional examinations in patients with typical LYP and absence of palpable enlarged lymph nodes, absence of hepatosplenomegaly, normal laboratory tests, and absence of B symptoms. 
        PCALCL: Contrast-enhanced CT scan with or without positron emission tomography (chest, abdomen, pelvis) or whole-body integrated positron emission tomography/CT. 
    Bone marrow aspirate or biopsy 
        LYP: Not performed in patients with typical LYP 
        PCALCL: Optional in patients with solitary PCALCL or patients with PCALCL without extracutaneous involvement in radiologic examinations (D)19  
        Lymph node biopsy: If enlarged lymph nodes (defined as > 1.5 cm in greatest transverse [long axis] diameter) are palpable or enlarged lymph nodes are detected on radiologic examination. 

Adapted from Bekkenk et al.

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