Successful treatment of leukaemia cutis with cladribine in a patient with B-cell chronic lymphocytic leukaemia

Cutaneous presentation of B-cell chronic lymphocytic leukaemia (B-CLL) is uncommon, and the influence of skin changes on B-CLL prognosis is unclear. We report a patient with B-CLL Rai II, with multiple nodular skin infiltrations on the trunk, upper arms and thighs as well as constitutional symptoms, who was successfully treated with cladribine. The peripheral blood (PB) lymphocytes were CD19, CD20, CD23 and CD5 positive, which confirmed the diagnosis of B-CLL. Skin biopsy of one of the lesions showed an intense infiltrate composed of small lymphocytes with no epidermotropism. These cells also showed the expression of CD19, CD20, CD23 and CD5 antigens similar to those presented on PB lymphocytes. Polymerase chain reaction performed on bone marrow lymphocytes and a lesional skin biopsy using consensus primers for immunoglobulin heavy-chain genes also showed the same monoclonal population of B lymphocytes both in the bone marrow and in the skin. The patient received four courses of cladribine 0.12 mg kg-1 daily as a 2-h infusion for five consecutive days. The courses were repeated at monthly intervals. The lymphocytosis gradually decreased and the PB count normalized after three courses. At the same time, a significant decrease in the cutaneous symptoms was observed. The patient became free of skin tumours after the fourth course of cladribine; only slight discoloration at the previous sites of cutaneous infiltration remained. There was no relapse of leukaemia cutis during a further 7 months of observation.     

Richter syndrome first manifesting as cutaneous B-cell lymphoma clonally distinct from primary B-cell chronic lymphocytic leukaemia

Richter syndrome (RS) is a transformation to high-grade non-Hodgkin lymphoma in patients with chronic lymphocytic leukaemia (CLL). RS may develop in lymph nodes or rarely extranodally. Skin localization of RS has been described in only a few cases. We present a 77-year-old woman who developed isolated diffuse large B-cell lymphoma (LBCL) in the skin of the nose without any other symptoms of RS. The LBCL in the skin was clonally distinct from the original bone marrow CLL cells. Moreover, LBCL cells were positive for LMP-1 segment of Epstein-Barr virus and overexpressed p53 protein. The patient was successfully treated with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) and adjuvant local radiotherapy.     

Primary cutaneous marginal zone B-cell lymphoma in a patient with chronic lymphocytic leukaemia

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade malignant lymphoma that presents in the skin with no evidence of extracutaneous localization at diagnosis. We present an 80-year-old woman with B-cell chronic lymphocytic leukaemia (CLL) who developed multifocal PCMZL lesions 14 months after CLL diagnosis. PCMZL was clonally similar to the original bone marrow (BM) CLL cells. The specific translocation t(14;18) (q32;q21) with breakpoints in IGH and BCL2 loci was found in a skin specimen, but was absent in BM and peripheral blood (PB) cells. In contrast, a 13q deletion was found in BM and PB CLL cells. The patient was treated with chlorambucil and complete response of PCMZL was achieved. To our knowledge this is the first patient with CLL in whom PCMZL has been diagnosed.     

Hypereosinophilic dermatitis-like erythema annulare centrifugum in a patient with chronic lymphocytic leukaemia

A 65-year-old male presented with a history of disseminated erythema annulare centrifugum lesions and a high peripheral blood eosinophilia (55%). Histopathology of a lesion revealed a superficial and deep perivascular and interstitial inflammatory eosinophilic infiltrate. The bone marrow aspirate showed a heavy eosinophilic infiltrate. There was no evidence of any other systemic involvement except for bone marrow eosinophilia. The patient had a 6-year history of chronic lymphocytic leukaemia. The disease is in complete remission for the last 3 years. The clinical and histopathological findings in the reported patient correspond to the diagnosis of hypereosinophilic dermatitis. Topical treatment with corticosteroid creams was successful. The cutaneous lesions gradually resolved in 8 weeks, within 7 months the peripheral blood eosinophilia slowly declined from 55% to 7%.     

Pre‐operative ultrasound with a 12–15 MHz linear probe reliably differentiates between melanoma thicker and thinner than 1 mm

Background Pre‐operative determination of primary melanoma thickness could be a tool to identify those patients who could be treated with radical primary tumour excision and sentinel lymph node biopsy in a single procedure. An excellent correlation between sonographic and histological measurement of maximal tumour thickness has been achieved using 20‐MHz transducers. Objective To show that widely available high resolution ultrasound with 12–15 MHz linear probe could also reliably assess the thickness of primary melanoma. Methods Sixty‐nine patients underwent ultrasound evaluation of 70 clinically and dermoscopically suspicious pigmented skin lesions before surgical excision. Results The sensitivity, specificity, positive and negative predictive values of ultrasound to detect melanoma > 1 mm were 92%, 92%, 95% and 81% respectively. The correlation between ultrasound and histological tumour thickness was very good [Pearson’s correlating index 0.823 (P < 0.001)]. Mean difference between sonographic and histological measurements was 0.045 mm with limits of agreement estimated at ?1.4 and +1.49, and a bias between two methods 45 μm. Conclusion Ultrasound examination with a 12–15 MHz linear transducer can reliably differentiate primary melanoma > 1 mm from those ≤ 1 mm.     

Fine-needle aspiration biopsy with ultrasound guidance in patients with malignant melanoma and palpable lymph nodes

Background Recurrence after treatment of stage I–II melanoma involves regional lymph nodes in about 50% of patients. A reliable method is needed to evaluate lymph node status (metastatic or not) in the case of palpable lymph nodes. Objectives To evaluate the efficiency of fine‐needle aspiration biopsy (FNAB) in examining clinically detected suspicious lymph node in patients followed up after surgical removal of stage I–II melanoma. Patients and methods One hundred and twenty FNABs were performed in 67 patients with a suspicious node in an open study conducted in a French melanoma regional referral centre, Hôpital de l'Hôtel‐Dieu. Cytodiagnosis was classified as positive, negative, inadequate or inconclusive. Sensitivity, specificity, positive and negative predictive values and positive and negative likelihood ratios were calculated after final histopathological evaluation. Results Fifty‐eight of 120 FNABs were positive (48%), 50 of 120 (42%) were negative, four of 120 (3%) were inconclusive and eight of 120 (7%) were inadequate. Among the 108 FNABs in which a definitive diagnosis could be given, sensitivity was 98·2% [95% confidence interval (CI) 90·7–99·9] and specificity was 96·1% (95% CI 86·8–98·9). Conclusions FNAB under ultrasound guidance is an efficient tool to discriminate better between cases in which surgical treatment of the lymph node basin should be performed and patients who should return for follow‐up. Surgical treatment appears to be required in cases of positive FNAB or in inconclusive cases.     

Giant orf in a patient with chronic lymphocytic leukaemia

A case of orf is presented in a patient with chronic lymphocytic leukaemia treated with chlorambucil and prednisone. Diagnosis was confirmed by electron microscopy and histopathology. Human orf is normally a self-limiting and benign condition but in this case the lesion grew to a size of 60 X 60 X 28 mm. When it was excised, local recurrence was treated with topical idoxuridine.     

Eosinophilic spongiosis in association with bullous pemphigoid and chronic lymphocytic leukaemia

We report the onset of an unusual blistering eruption following a diagnosis of B-cell chronic lymphocytic leukaemia (CLL). The histology consistently showed eosinophilic spongiosis, but the diagnosis of bullous pemphigoid was only confirmed after 13 years by repeated immunofluorescence studies. The occurrence of subepidermal blistering diseases in association with B-cell lymphoproliferative disorders is rare; a recent study showed that the majority of these cases are epidermolysis bullosa acquisita, confirmed by immunological studies. Only two cases of immunofluorescence-proven bullous pemphigoid in association with CLL have been previously reported.     

Skin involvement in chronic myelomonocytic leukaemia as a predictor of transformation into acute myeloid leukaemia.

We report on the case of a patient with myelodysplastic syndrome (MDS) who presented with leukaemia cutis preceding development of acute myeloid leukaemia. Leukaemic infiltration of the skin should be considered an early manifestation of leukaemic transformation and an indicator of poor prognosis in MDS.     

Two cases of merkel cell tumour arising in patients with chronic lymphocytic leukaemia

Chronic lymphocytic leukaemia (CLL) has been associated with an increased incidence of second neoplasms, especially skin cancers such as basal and squamous cell carcinomas. No association with the rarer skin cancer, the Merkel cell tumour (MCT), has previously been reported. Two patients with MCT had a previous diagnosis of CLL. MCT is an aggressive skin cancer, as up to 45% of patients have lymph node involvement at presentation and 5-year disease-free survival is as low as 30%. It is most commonly found on sun-exposed areas of the body, and ultraviolet radiation together with drug-induced or CLL-induced immunosuppression may be the underlying mechanism in the observed relationship between CLL and other skin cancers.     

Bilateral areolar leiomyomas in a patient undergoing BRAF inhibition therapy for melanoma

BRAF inhibition therapy, used to treat melanomas with BRAF mutations, is associated with both neoplastic and non‐neoplastic cutaneous side effects including squamous cell carcinomas, warty dyskeratomas, verrucous keratoses, photosensitivity and widespread eruptions that present histopathologically as acantholytic dyskeratosis. We report a case of a patient undergoing BRAF inhibition therapy for disseminated melanoma with a V600E mutation who developed bilateral areolar leiomyomas, one of which was biopsied and the other of which resolved after discontinuation of vemurafenib therapy. To our knowledge, this is the first reported case of a mesenchymal neoplasm developing in association with BRAF inhibition therapy.     

Progressive trichodysplasia spinulosa in a patient with chronic lymphocytic leukaemia in remission

A 70-year old Caucasian man with chronic lymphocytic leukaemia developed trichodysplasia spinulosa 2 months after ceasing chemotherapy. Histological features characteristic to this condition include dilated and enlarged hair follicles, hyperplastic hair bulbs, hyperplasia of inner root sheath cells with numerous large, eosinophilic, trichohyaline granules, and hypercornification. Although he was in remission for chronic lymphocytic leukaemia, lesions were slowly progressive 15 months after cessation of chemotherapy. We also describe a painless pull-test where spicules can be easily plucked and assessed microscopically for inner root sheath keratinization, or observed with surface microscopy in a clinic setting.