TABLE 1.
Demographic, clinicopathologic and molecular features, treatment and outcome in patients with LCH and lymphomas
| Case | Age | Sex | WBC (x 103/μ l) |
Hgb (g/d L) |
Plt (x 103/ μl) |
LDH (IU/L ) |
B2M (mg/ L) |
Anatomic location |
Lymphoma subtype |
LCH distribution |
LCH component IHC |
pERK IHC |
BRAF V600E IHC |
BRAF V600E PCR |
MAP2K1 PCR |
Treatment | Follow up (months) |
Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 68 | F | N/A | N/A | N/A | N/A | N/A | Lymph node, cervical | AITL | Multiple small foci | CD1a + langerin + S100 + | Pos | Neg | Neg | Neg | N/A | N/A | N/A |
| 2 | 84 | F | 1 0.5 | 13.2 | 368 | 397 | 2 | Lymph node, cervical | CHL, mixed cellularity | Multiple small foci and scattered LCs | CD1a + langerin + S100 + | Neg | Neg | Neg | Neg | AVD XRT |
10 | CR |
| 3 | 28 | M | 6.6 | 16.2 | 154 | 465 | 2.8 | Chest wall | CHL, nodular sclerosis | Few small foci | CD1a + langerin + S100 + | Neg | Neg | Neg | Neg | ABVD | 67 | CR |
| 4 | 37 | M | 4.0 | 9.8 | 41 | 668 | 2.4 | Lymph node, axillary | CHL, nodular sclerosis | Multiple small foci, 2 large focus | CD1a + langerin + S100 + | Pos in subcap sular foci; neg in largest focus | Neg | Neg | Neg | ABVD, VDTPACE, IGEV, thalidomide, revlimid, XRT, auto-SCT | 21 | DOD (CHL) |
| 5 | 39 | F | 5.3 | 15.2 | 162 | 586 | 2.1 | Lymph node, cervical | CHL, nodular sclerosis | Multifocal, large clusters of LCs | CD1a + langerin + S100 + | Neg | Pos | Neg | Neg | ABVD, AVD, ICE 1st cycle, will be followed by SCT | 6 | Persistent disease in the mediastinum detected by imaging |
| 6 | 54 | M | 5.8 | 12.1 | 141 | 402 | 4.3 | Lymph node, retroperitone al | CHL, recurrent | Small cluster with central necrosis | CD1a + langerin + S100 + | Pos | Neg | Neg | Neg | ABVD, ICE, auto-SCT for CHL Azacitidine, decitabine, MUD-SCT for MDS |
21 | Therapy-relatedMDS |
| 7 | 61 | M | 13.2 | 13.4 | 344 | 390 | 1.8 | Lymph node, inguinal | MCL | Small cluster | CD1a + langerin + S100 + | Neg | Neg | Neg | Neg | Rituximab, hyper CVAD, methotrexate, cytarabine | 89 | CR |
Abbreviations: LCH, Langerhans cell histiocytosis; WBC, white blood cells; Hgb, hemoglobin; Plt, platelets; LDH, lactate dehydrogenase; B2M, β-2 microglobulin; IHC, immunohistochemistry; pERK, phosphorylated ERK; PCR, polymerase chain reaction; F, female; M, male; N/A, not available; AITL, angioimmunoblastic T-cell lymphoma; CHL, classical Hodgkin lymphoma; MCL, mantle cell lymphoma; LCs, Langerhans cells; Pos, positive; Neg, negative; AVD, doxorubicin, vinblastine and dacarbazine; XRT, radiotherapy; ABVD, doxorubicin, bleomycin, vinblastine and dacarbazine; VDTPACE, dexamethasone, thalidomide, cisplatin, doxorubicin, cyclophosphamide and etoposide; IGEV, ifosfamide, gemcitabine and vinorelbine; auto-SCT, autologous stem cell transplant; ICE, ifosfamide, carboplatin and etoposide; MUD-SCT, matched unrelated donor stem cell transplant; MDS, myelodysplastic syndrome; hyper CVAD, cyclophosphamide, vincristine, doxorubicin, and dexamethasone; CR, complete remission; DOD, died of disease