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Mantle cell lymphoma with skin involvement.

Kalińska-Bienias A, Ziarkiewicz-Wróblewska B, Kowalewski C, Woźniak K - Postepy Dermatol Alergol (2015)

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland. Head of the Department: Prof. Cezary Kowalewski MD, PhD.

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Mantle cell lymphoma (MCL) is a rare disease of the lymphomatoid system arising from mature B lymphocytes and comprises 3–10% of all non-Hodgkin's lymphoma subtypes and typically involves lymph nodes... According to the guidelines of the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC), the diagnosis of MCL should be established on the basis of morphological examination and immunophenotyping with detection of cyclin D1 protein overexpression and/or chromosomal translocation t(11,14)(q13;q32) of the CCND1 gene... Mantle cell lymphoma was diagnosed in a skin biopsy specimen, lymph node and bone marrow... On the basis of the histological and immunohistochemical data, the diagnosis of MCL was made and in the staging evaluation the patient presented with Ann Arbor stage IV D/B with lymphadenopathy and the bone marrow involvement... After five cycles of the chemotherapy the patient achieved a partial remission of skin lesions and lymphadenopathy and complete remission of dyspnea... At that time her general condition improved... After 7 months there was the recurrence of the disease and despite receiving aggressive multiagent chemotherapy DHAP (dexamethasone, cytarabine, cisplatin), the chemo-resistance was present and the patient died after several weeks (1 year after diagnosis of MCL)... It seems to be over-reactive rather than malignancy because the tumor cells were not detected in these cases... Our patient presented neither clinically nor histopathologically skin symptoms typical of an insect bite... In MCL, the expression of cyclin D1is rather diffuse and comprises almost all tumor cells as distinct from CLL/SLL in which only the lymphoproliferative central zone is expressed... To differentiate between BCLL/SLL and MCL overexpression of CD23 is proved to be important... In our case the results of negative CD23 staining in the lymph node and positive cyclin D1 make it possible to exclude of BCLL/SLL... In reported cases, 11 patients achieved a complete remission and no recurrence was observed during a follow-up period... In conclusion, MCL is a rare lymphoma and if skin is involved this always suggests dissemination of the disease.

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Related in: MedlinePlus

Histological and immunohistochemical findings of the patient's skin (A–C), lymph node (D–F) and bone marrow (G–H): A – proliferation of small to medium-sized atypical lymphoid cells (hematoxylin-eosin stain, 100×), B – expression of CD20 by neoplastic cells (100×), C – no expression of CD3 (100×), D – diffuse proliferation of lymphoid cells (hematoxylin- eosin stain, 200×), E – expression of CD5 by neoplastic cells (200×), F – staining with antibody to Ki 67 (200×), G – diffuse proliferation of lymphoid cells (hematoxylin-eosin stain, 200×), H – neoplastic cells expressed cyclin D1 (200×)
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Figure 0002: Histological and immunohistochemical findings of the patient's skin (A–C), lymph node (D–F) and bone marrow (G–H): A – proliferation of small to medium-sized atypical lymphoid cells (hematoxylin-eosin stain, 100×), B – expression of CD20 by neoplastic cells (100×), C – no expression of CD3 (100×), D – diffuse proliferation of lymphoid cells (hematoxylin- eosin stain, 200×), E – expression of CD5 by neoplastic cells (200×), F – staining with antibody to Ki 67 (200×), G – diffuse proliferation of lymphoid cells (hematoxylin-eosin stain, 200×), H – neoplastic cells expressed cyclin D1 (200×)

Mentions: A 63-year-old woman referred to the Department of Dermatology presented with severe thick erythematous infiltrations located on all her face and the ears as well as hyperpigmentations on the upper part of the trunk. Skin lesions on the face started as erythema resembling photosensitivity and progressively enlarged (Figures 1 A–B). On admission the patient has had the dryness of the oral mucosa, difficulties in speaking, increased dyspnea for several weeks. The generalized lymphadenopathy was observed. Laboratory abnormalities included an elevated level of leucocyte count of 24 000/mm3 with lymphocytosis of 75% and anemia with hemoglobin of 7.7 g/dl. Mantle cell lymphoma was diagnosed in a skin biopsy specimen, lymph node and bone marrow. Skin biopsy taken from the ear showed a numerous diffuse lymphoid infiltrate in dermal and subcutaneous tissue with a grenz zone with sparing the epidermis. Neoplastic cells were intermediate to large size with irregular and hyperchromatic nuclei with increased mitotic activity (Figure 2 A). Histopathological examination taken from the lymph node showed numerous lymphoid infiltrates (Figure 2 D) and histopathology from the bone narrow revealed infiltration of lymphoma covered more than a half of the tissue of bone marrow (Figure 2 G). Immunohistochemically, the tumor cells from the skin, lymph node and bone marrow were positive for CD20, CD5 and were negative for CD3 marker (Figures 2 B, C, E). In the lymph node and bone marrow, CD23 staining was negative (figure not shown). The proliferation index measured by immunostaining with MIB1 was positive in 50% of the cells of the lymph node (Figure 2 F). Immunophenotypic analysis by flow cytometry on bone marrow aspirate specimens showed the presence of CD5, CD19, CD20 markers on over 90% of the cells and clonal expression of immunoglobulin κ and λ light chains. Cyclin D1 was strongly expressed (Figure 2 H). Computed tomography (CT) scans of the neck and chest showed a tumor lesion in the larynx and enlarged lymph nodes along cervical vessels and the package of lymph nodes in the mediastinum. Computed tomography (CT) scans of the abdomen and pelvis revealed enlarged periaortic and inguinal lymph nodes as well as in the mesentery of the intestine. On the basis of the histological and immunohistochemical data, the diagnosis of MCL was made and in the staging evaluation the patient presented with Ann Arbor stage IV D/B with lymphadenopathy and the bone marrow involvement. The patient was referred to the Hematology Department, where she received the CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy. After five cycles of the chemotherapy the patient achieved a partial remission of skin lesions and lymphadenopathy and complete remission of dyspnea. At that time her general condition improved. After 7 months there was the recurrence of the disease and despite receiving aggressive multiagent chemotherapy DHAP (dexamethasone, cytarabine, cisplatin), the chemo-resistance was present and the patient died after several weeks (1 year after diagnosis of MCL).


Mantle cell lymphoma with skin involvement.

Kalińska-Bienias A, Ziarkiewicz-Wróblewska B, Kowalewski C, Woźniak K - Postepy Dermatol Alergol (2015)

Histological and immunohistochemical findings of the patient's skin (A–C), lymph node (D–F) and bone marrow (G–H): A – proliferation of small to medium-sized atypical lymphoid cells (hematoxylin-eosin stain, 100×), B – expression of CD20 by neoplastic cells (100×), C – no expression of CD3 (100×), D – diffuse proliferation of lymphoid cells (hematoxylin- eosin stain, 200×), E – expression of CD5 by neoplastic cells (200×), F – staining with antibody to Ki 67 (200×), G – diffuse proliferation of lymphoid cells (hematoxylin-eosin stain, 200×), H – neoplastic cells expressed cyclin D1 (200×)
© Copyright Policy - open-access
Related In: Results  -  Collection

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Show All Figures
getmorefigures.php?uid=PMC4495111&req=5

Figure 0002: Histological and immunohistochemical findings of the patient's skin (A–C), lymph node (D–F) and bone marrow (G–H): A – proliferation of small to medium-sized atypical lymphoid cells (hematoxylin-eosin stain, 100×), B – expression of CD20 by neoplastic cells (100×), C – no expression of CD3 (100×), D – diffuse proliferation of lymphoid cells (hematoxylin- eosin stain, 200×), E – expression of CD5 by neoplastic cells (200×), F – staining with antibody to Ki 67 (200×), G – diffuse proliferation of lymphoid cells (hematoxylin-eosin stain, 200×), H – neoplastic cells expressed cyclin D1 (200×)
Mentions: A 63-year-old woman referred to the Department of Dermatology presented with severe thick erythematous infiltrations located on all her face and the ears as well as hyperpigmentations on the upper part of the trunk. Skin lesions on the face started as erythema resembling photosensitivity and progressively enlarged (Figures 1 A–B). On admission the patient has had the dryness of the oral mucosa, difficulties in speaking, increased dyspnea for several weeks. The generalized lymphadenopathy was observed. Laboratory abnormalities included an elevated level of leucocyte count of 24 000/mm3 with lymphocytosis of 75% and anemia with hemoglobin of 7.7 g/dl. Mantle cell lymphoma was diagnosed in a skin biopsy specimen, lymph node and bone marrow. Skin biopsy taken from the ear showed a numerous diffuse lymphoid infiltrate in dermal and subcutaneous tissue with a grenz zone with sparing the epidermis. Neoplastic cells were intermediate to large size with irregular and hyperchromatic nuclei with increased mitotic activity (Figure 2 A). Histopathological examination taken from the lymph node showed numerous lymphoid infiltrates (Figure 2 D) and histopathology from the bone narrow revealed infiltration of lymphoma covered more than a half of the tissue of bone marrow (Figure 2 G). Immunohistochemically, the tumor cells from the skin, lymph node and bone marrow were positive for CD20, CD5 and were negative for CD3 marker (Figures 2 B, C, E). In the lymph node and bone marrow, CD23 staining was negative (figure not shown). The proliferation index measured by immunostaining with MIB1 was positive in 50% of the cells of the lymph node (Figure 2 F). Immunophenotypic analysis by flow cytometry on bone marrow aspirate specimens showed the presence of CD5, CD19, CD20 markers on over 90% of the cells and clonal expression of immunoglobulin κ and λ light chains. Cyclin D1 was strongly expressed (Figure 2 H). Computed tomography (CT) scans of the neck and chest showed a tumor lesion in the larynx and enlarged lymph nodes along cervical vessels and the package of lymph nodes in the mediastinum. Computed tomography (CT) scans of the abdomen and pelvis revealed enlarged periaortic and inguinal lymph nodes as well as in the mesentery of the intestine. On the basis of the histological and immunohistochemical data, the diagnosis of MCL was made and in the staging evaluation the patient presented with Ann Arbor stage IV D/B with lymphadenopathy and the bone marrow involvement. The patient was referred to the Hematology Department, where she received the CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy. After five cycles of the chemotherapy the patient achieved a partial remission of skin lesions and lymphadenopathy and complete remission of dyspnea. At that time her general condition improved. After 7 months there was the recurrence of the disease and despite receiving aggressive multiagent chemotherapy DHAP (dexamethasone, cytarabine, cisplatin), the chemo-resistance was present and the patient died after several weeks (1 year after diagnosis of MCL).

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland. Head of the Department: Prof. Cezary Kowalewski MD, PhD.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Mantle cell lymphoma (MCL) is a rare disease of the lymphomatoid system arising from mature B lymphocytes and comprises 3–10% of all non-Hodgkin's lymphoma subtypes and typically involves lymph nodes... According to the guidelines of the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC), the diagnosis of MCL should be established on the basis of morphological examination and immunophenotyping with detection of cyclin D1 protein overexpression and/or chromosomal translocation t(11,14)(q13;q32) of the CCND1 gene... Mantle cell lymphoma was diagnosed in a skin biopsy specimen, lymph node and bone marrow... On the basis of the histological and immunohistochemical data, the diagnosis of MCL was made and in the staging evaluation the patient presented with Ann Arbor stage IV D/B with lymphadenopathy and the bone marrow involvement... After five cycles of the chemotherapy the patient achieved a partial remission of skin lesions and lymphadenopathy and complete remission of dyspnea... At that time her general condition improved... After 7 months there was the recurrence of the disease and despite receiving aggressive multiagent chemotherapy DHAP (dexamethasone, cytarabine, cisplatin), the chemo-resistance was present and the patient died after several weeks (1 year after diagnosis of MCL)... It seems to be over-reactive rather than malignancy because the tumor cells were not detected in these cases... Our patient presented neither clinically nor histopathologically skin symptoms typical of an insect bite... In MCL, the expression of cyclin D1is rather diffuse and comprises almost all tumor cells as distinct from CLL/SLL in which only the lymphoproliferative central zone is expressed... To differentiate between BCLL/SLL and MCL overexpression of CD23 is proved to be important... In our case the results of negative CD23 staining in the lymph node and positive cyclin D1 make it possible to exclude of BCLL/SLL... In reported cases, 11 patients achieved a complete remission and no recurrence was observed during a follow-up period... In conclusion, MCL is a rare lymphoma and if skin is involved this always suggests dissemination of the disease.

No MeSH data available.


Related in: MedlinePlus