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.4