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Table 1 Clinical presentation, treatment and outcome of the 20 subcutaneous panniculitis-like T-cell lymphoma (SPTL) patients studied

From: Molecular characterization of subcutaneous panniculitis-like T-cell lymphoma reveals upregulation of immunosuppression- and autoimmunity-associated genes

Case Sex and age* First clinical presentation Clinical picture, concomitant diseases* Abnormal laboratory findings Therapy Outcome PE
1 F15 In 2006 first sub-cutaneous lesion laterally on trunk, a year later ca. 50 widespread subcutaneous nodules, SPTL diagnosis in 05/2008 No B-symptoms CT normal, mild splenomegaly (diam. 13 cm) 06/2008 prednisolone CR since 06/2010; 07/2011 a solitary MF lesion  
Otherwise healthy No ANA/ ENA/DNA antibodies 60 mg/d (2 weeks), slowly tapering until 03/2009, and 09/2008 MTX 10 mg/wk until 02/2010 → CR
TCR clonality + in skin lesion (2008), LD marginally elevated 232 U/l (normal range 115–235 U/l) at the time of diagnosis relapse 05/2010 → MTX 7,5 mg/wk → CR
2 M27 (case 8 in [5] Subcutaneous nodules in forehead and scalp (horse shoe shape) in 2005, SPTL diagnosis 12/2006 No B-symptoms Cervical, thoracic and abdominal CT normal 01/2007 prednisolone CR since 02/2008  
Cervical lymph nodes enlarged (since mononucleosis years earlier ) BM normal 60 mg/d (2 weeks) for 3 weeks → CR
→ reactive histology ANA-ab 320 (centromeric staining), ENA-ab positive but specific RNP-, SSA-, SSB- and Sm-abs negative 04/2007 prednisolone
80 mg/d until 01/2008 → CR
3 F66 09/2003 small, reddish papules in upper and lower extremities and nodules on shoulders; histology in 2004: lupus erythematosus profundus; treated with hydroxychloroquiine 300 mg/d (06-12/2005), dapsone 50 mg/d (01-04/2008) with no response Hypertension, dyslipidemy Thrombocytosis, leukopenia since 1999 → BM normal, chromosomes normal 05/2008 prednisolone CR since 03/2011  
Photosensitivity → photoprovocation negative → no specific diagnosis 60 mg/d (2 weeks) decreasing until 12/2008 → CR, relapse 01/2009 – 06/2009 MTX10 mg/wk, prednisolone 40 mg /d (2 weeks) decreasing until 04/2010 → CR, relapsing disease
Low CD4 levels in 2008: 0,065 - 0,150/17-23% (normal range 0,458-1,406 E9/l/29-59%) → sulfatrimetoprim prophylaxis 12/2009 – 02/2011 bexarotene 225 mg/d** → initially PR, then PD
SPTL diagnosis in 05/2008   No ANA, ENA or DNA antibodies 02/2011 prednisolone 10 mg/d + MTX 5 mg/wk maintenance → CR
TCR clonality + in blood (07/2008) and in MB (12/2009), LD slightly elevated 248–380 U/l in 05/2009 – 01/2011
CT normal
4 M 47 Subcutaneous tender nodules on buttock (15 cm) and trunk in 10/2013, SPTL diagnosed in 2/2014 Fatigue, daily fever up to 38,5C, cough, joint pains; no concomitant diseases WBC 2,6 x 109/l, B-Ly 0,88 x 109/l (33%), B-T-CD4 0,271 x 109/l, CRP 11, total ENA abs 1,6 (ref. <0,7) Prednisolone 80 mg/d for one week, then 50 mg/d for 1,5 months CCR after 3 months, all laboratory values normalized (ALT 79), patient returned back to work  
BM normal, TCR clonality in lymph node, ALP 109, ALT 545, LD 1162 U/l, CT : few 1 cm lymph nodes in right axilla and left inguinal, liver slightly enlarged 16 cm - > 30 mg/d
5 F 60 case 7 in [5] Subcutaneous, firm nodules on fore-head (1 cm) and on upper extremities (blueish) in 2005; SPTL diagnosis in 12/2006 No B-symptoms, no enlarged lymph nodes CBC normal, LD ad 299 U/l until 06/2009, thereafter normalized 03/2007 EB therapy → PR, 06/2007 prednisolone 60 mg/d (2 weeks) decreasing doses until 11/07 → CR PR  
Psoriatic arthropathy treated with leflunomide 20 mg/d 05/2003 – 12/2006 No ANA, ENA or DNA abs, M-component in serum, decreased during follow-up 03/2008 prednisolone
Hypertension Dyslipidemy Thoracic and cervical CT normal 60 mg/d (2 weeks) decreasing doses until 12/2009 → PR
12/2008 MTX 7,5 mg – 12,5 mg/wk → stopped 10/2010 → PR
10/2010 bexarotene
225 mg/d → stopped 02/2011 (ALT elevation 110 U/l) → initially PR then PD
05-06/2011 EB therapy → SD, 08-11/2011 CHOP → PR
6 F39 Subcutaneous, firm nodules in upper and lower extremities, and trunk in 2008; 2/2010 lobular panniculitis in biopsy, 5/2011 SPTL histologically No B-symptoms, no enlarged lymph nodes; some joint pains concomitantly, hypothyreosis since years CBCnormal; no ANA, ENA, DNA , TPO or Thygl abs, RF 59 IU/ml (reference 0–14), LD normal; CT normal 8/2011 prednisolone 20– 60 mg/d → CR , relapse in 8/2012, whereafter prednisolone 15 mg/d + MTX 12,5 mg/wk → CR CR in 1/2013  
7 F14 case 9 in [5] Subcutaneous nodules in abdominal region, lower and upper extremities in 01/2006, SPTL diagnosis in 02/2007 No B-symptoms, no enlarged lymph nodes CBC normal at the time of diagnosis, thereafter mild anemia (Hb 110–120 g/l) 02/2007 prednisolone CR since 09/2009  
Atopic constitution No ANA, ENA or DNA antibodies 40 mg/d for 1,5 months → CR (thereafter spontaneously resolving lesions)
LD slightly elevated (242) U/l) at the time of diagnosis, thereafter normalized
Thorax CT normal  
8 F61 SPTL diagnosis in 03/2001, involvement of both lower legs Multiple analgetic intolerance LD slightly elevated 236 U/l Initial therapy: radiation therapy and CHOP 6 cycles → CR, relapse in 2004, bexarotene and steroids from 2004 → PR/SD SD  
9 F77 SPTL diagnosis in 09/2007, involvement of extremities and thoracic area Liver cysts, uterine myoma LD slightly elevated 259 U/l Encapsulated doxorubicin 8 cycles + prednisolone → PR DOD 05/2009  
IFNα 3 x 6 mio
Radiation right thigh 36 Gy
Thoracic lesions: triamcinolone intralesional
14 F68 07/2011, two nodular lesions of the lower limb and a nasal tumor. SPTL confirmed 09/2011 Weight loss, asthenia and fever. Splenomegaly and adenopathies. history of vasculitis between 1994 and 2007 Cytopenia (lymphopenia and thrombocytopenia), elevated liver enzymes, LD >2 N, b2microglobulin 7 mg/L Chemotherapy with etoposide (08/2011), followed by CHEP (09/2011-10/2011) and CHOP (11/2011-02/2012). CR since 03/2012.  
15 M*** [6] In 02/2007, three plaques on the left and right upper limb (5-10 cm), and face; diagnosis confirmed in 06/2007 Fever, asthenia, hepatomegaly and splenomegaly, macrophage activation syndrome LD >2 N, elevated liver enzymes, ANA and anti SSA +, TCR clonality + . Corticosteroid and cyclosporine A (2007–2010) CR in 03/2008 without relapse.  
16 F22 Medical history of cytophagic and histiocytic panniculitis in 1993, treated with corticosteroid. SPTL diagnosed in 2000, widespread plaque and nodule lesions on upper and lower limbs, trunk and face. Fever, weight loss, asthenia, hepatomegaly and splenomegaly. Macrophage activation syndrome. LD >2 N. No ANA abs, b2microglobulin >7 mg/L Chemotherapy with autologous stem cell transplantation in 2001, relapse in 2001. Corticosteroid and MTX between 2002 and 2008 - > CR CR in 11/2002, no relapse.  
17 F37 EN in 2010. In 01/2011, 8 nodules on trunk and face. SPTL diagnosed in 04/2011. Asthenia, myalgia and diarrhea. Lymphopenia, No ANA abs, TCR clonality + Hydroxychloroquine in 07/2011 CR in 10/2011, no relapse.  
18 M48 AL amyloidosis in 07/2007 (chemotherapy + autologous stem cell transplantation). In 04/2011, 3 nodules (lower limb,trunk). SPTL confirmed in 09/2011 Adenopathies, hepatomegaly and splenomegaly Leukopenia, anemia, thrombocytopenia, TCR clonality + Cyclophosphamide, adriamycin, vincristine and methylprednisolone in 09/2011. PR, deceased in 04/2012 (infectious pneumopathy)  
19 F50 Multiple nodules of the upper and lower limbs and trunk in 01/2011. SPTL confirmed in 02/2011 - Elevated liver enzyme and LD. Corticosteroid in 04/2011 CR in 07/2011  
20 F15 One isolated nodule (>10 cm) of the lower limb in 12/2011. SPTL confirmed in 04/2012 Fever, asthenia, weight loss, adenopathy Leucopenia, lymphopenia, elevated LDH. TCR clonality + Corticosteroid (05-06/2012), vinblastine 05/2012), multiple courses of chemotherapy since 06/2012 PR in 01/2013  
  1. (Clinical details of cases 10–13, all in remission, are reported in [5], in Table 1 as cases 3–6).
  2. =*at the time of diagnosis.
  3. **Since Mehta and coworkers recently reported promising results of bexarotene therapy in SPTL, [7], bexarotene was used for the treatment of three of our patients: cases 3, 5 and 7. Only case 7 remained in long-lasting remission with a combination therapy of bexarotene and steroids, and cases 3 and 5 reached only a temporary initial partial response [5].
  4. ***Case 15 reported first in [6] at the age of 9 months.
  5. CT = computed tomography, LD = lactate dehydrogenase, mtx = methotrexate, CR = complete remission, PR = partial remission, MF = mycosis fungoides, ANA = antinuclear antibody, ENA = extractable nuclear antigen, BM = bone marrow, PD = progressing disease, SD = stable disease, CBC = complete blood count, WBC = white blood cells, RBC = red blood cells, ALP = alkaline phosphatase, ALT = alanine aminotransferase, EB = electron beam radiation therapy, CHOP = cyclophosphamide, doxorubisine, oncovine, prednisolone, IFN = interferon, DOD = died of disease.