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