2014 Kempf Primary Cutaneous Follicle Cell Lymphoma

    Multiple choice question

    Question prepared using materials provided by Dr. Werner Kempf, M.D


    Which statement on primary cutaneous follicle center lymphoma (PCFCL) is NOT correct?

    2014 Kempf Follicular Helper T-Cells

    Multiple choice question

    Question prepared using materials provided by Dr. Werner Kempf, M.D


    Which statement regarding follicular helper T-cells (FHT) is NOT correct?

    2014 Kempf Lymphomatoid Papulosis

    Multiple choice question

    Question prepared using materials provided by Dr. Werner Kempf, M.D


    Which statement regarding lymphomatoid papulosis is correct?

    2014 Kempf Anaplastic Large Cell Lymphoma

    Multiple choice question

    Question prepared using materials provided by Dr. Werner Kempf, M.D

    Which statement regarding primary cutaneous CD30+ anaplastic large cell lymphoma (ALCL) is NOT correct?

    2014 Zembowicz Rosai-Dorfman

    Multiple choice question

    Question prepared using materials provided by Dr. Artur Zembowicz, M.D, Ph.D.

    The biopsy below is from a 43 year old Asian-American female with a 4 month history of brownish dermal nodule on the chest. The biopsy shows the typical features of Rosai-Dorfman disease.

    Which of the following statements regarding this disease is correct?

    2014 Zembowicz Necrobiotic Xanthogranuloma

    Multiple choice question

    Question prepared using materials provided by Dr. Artur Zembowicz, M.D, Ph.D.

    A 68 year old male with a past medical history of multiple myeloma associated with a paraproteinemia presents with multiple, crusted, red plaques on the scalp and torso. A biopsy was performed.


    What is the best diagnosis?

    2014 Zembowicz Primary Cutaneous Large B-cell Lymphoma, Leg Type

    Multiple choice question

    Question prepared using materials provided by Dr. Artur Zembowicz, M.D, Ph.D.

     

    The expected immunophenotype of primary cutaneous large B-cell lymphoma, leg type is?

    2014 Zembowicz Diffuse Large B-Cell Lymphoma

    Multiple choice question

    Question prepared using materials provided by Dr. Artur Zembowicz, M.D, Ph.D.

    The biopsy illustrated below is from an 84 year old female with a history of breast cancer presenting with a subcutaneous nodules on the left hip. Morphology of the lesion is consistent with diffuse large cell lymphoma. The lesion shows expression of CD20, MUM1, and Bcl2. It is negative for CD10 and Bcl6.

    Which statement regarding this case is NOT correct?

    2014 Zembowicz Sezary Syndrome

    Multiple choice question

    Question prepared using materials provided by Dr. Artur Zembowicz, M.D, Ph.D.

    The biopsy below is from a case of a patient presenting with erythroderma. The patient was treated for suspected eczema. As clinical response was limited a biopsy was performed to rule out Sezary Syndrome. The biopsy showed spongiotic dermatitis.



    Which of the following statements about this case is correct?

    2014 Zembowicz Follicular Mycosis Fungoides

    Multiple choice question

    Question prepared using materials provided by Dr. Artur Zembowicz, M.D, Ph.D.

    The slide below depicts subsets of mycosis fungoides recognized by the 2008 WHO subsets. D’emblee mycosis fungoides is no longer a recognized category and cutaneous T-cell lymphomas presenting de novo as plaques or tumors represent another category of T-cell lymphoma. Granulomatous mycosis fungoides is associated with a worse prognosis.

     

    What is the reason that follicular mycosis fungoides is separated?

    2014 Liu Indolent CD8-positive T Cell Lymphoid Proliferation

    Multiple choice question

    Question prepared using materials provided by Dr. Vincent Liu, M.D

    A 55 year old white male presents with a 1 cm skin-colored nodule on the right ear helix. It has been present for 2 years. The patient has a past medical history of diabetes mellitus and hypertension. A ROS is negative. A punch biopsy is done and shown below.



    What is the most likely diagnosis?

    2014 Liu Lymphoplasmacytic Plaque of Childhood

    Multiple choice question

    Question prepared using materials provided by Dr. Vincent Liu, M.D

    A 12 year old white female presents with an asymptomatic, erythematous 2cm plaque. It has been present on the left pretibial leg for one year. There has only been a slight improvement with application of a mid-potency topical steroid. A biopsy is done with a clinical impression of a spitz nevus vs. a hemangioma. It shows plasma cell-rich lymphocytic infiltrate. The punch biopsy is shown below.

    What is the most likely diagnosis?

    2014 Kempf Primary Cutaneous Follicle Center Lymphoma

    Multiple choice question

    Question prepared using materials provided by Dr. Werner Kempf, M.D


    A 57 year old male presents with a new growing nodule on the scalp. The lesion has been present for 4 months. The patient denies any other lesions, fevers, chills, night sweats, or a history of lymphoma. On exam is the lesion pictured below.


    Lymph node examination shows nothing palpable. A biopsy is done is the histology is shown next.


    Histology shows a superficial and deep nodular infiltrate with follicle formation.


    The follicles are composed mostly of centrocytes with some centroblasts. There is a lack of tingle body macrophages.


    Bcl-6 staining if positive in the follicles.


    Bcl-2 staining of the centroblasts is negative.

    What is the best diagnosis?

    2014 Guitart Primary Cutaneous Marginal Zone Lymphoma

    Multiple choice question

    Question prepared using materials provided by Dr. Joan Guitart, M.D


    Presented are classic features of marginal zone lymphoma found in the skin.


    Nodular pattern of infiltrate. Lighter and darker areas of zoning.


    Atrophic remnants of germinal centers.

    What feature would favor a primary cutaneous marginal zone lymphoma from a systemic marginal zone lymphoma with secondary metastasis to the skin?

    2014 Guitart Aggressive Epidermotropic CD8+ T-Cell Lymphoma

    Multiple choice question

    Question prepared using materials provided by Dr. Joan Guitart, M.D

    A 56 year old women presents with new onset ulcerative plaques of 3 months duration. The plaques have rapidly spread and no treatment has been done since this is the first time the patient was able to see a doctor for treatment. The patient has admitted to have repeated fevers at night and waking up covered in sweat. The reporting doctor also says that she has bilateral lymphadenopathy in the axillae. A skin biopsy of one of the lesions is performed. Histology shows an acanthotic epidermis that is filled with atypical lymphocytes and involvement of hair follicles and eccrine glands. The atypical cells are monomorphic and there are scattered apoptotic keratinocytes. Immunohistochemistry shows that the cells are TIA-1, CD3, CD8, CD45RA, and CD7 positive. CD2, CD4, CD5, CD30, and CD56 are negative. TCR for γδ are negative.

    Multiple, widespread ulcerations and nodules.


    Epidermal involvement by somewhat monomorphous, atypical lymphocytes.


    Deeper nodular infiltrates with involvement of the eccrine gland pictured.

    CD8 immunohistochemistry showing positive staining of lymphocytes in the epidermis.

    What is the best diagnosis?

    2014 Wu Hydroa Vacciniforme-like Lymphoma

    Multiple choice question

    Question prepared using materials provided by Dr. Yu-Hung Wu, M.D.

    A 13 year old male patient from Taiwan presents with new onset necrotic papules on the cheeks. A biopsy is done and the pathology is shown below.

    Clinically necrotic papules and small ulcerations on the head.


    Low power view showing nodules of an infiltrate with some epidermal changes.

    Involvement of hair follicles and eccrine glands by a predominantly lymphocytic infiltrate.


    Angiocentricity of the lymphocytes in the deeper dermis.

    Immunohistochemistry shows that CD3, CD8, and TIA-1 are positive. CD4, CD56, and CD30 are negative. The findings are consistent with a diagnosis of hydroa vacciniforme-like lymphoma. With which virus is this lymphoma is associated with?

    2014 Kempf Cutaneous CD4 Small/Medium sized T-Cell Lymphomas

    Multiple choice question

    Question prepared using materials provided by Dr. Werner Kempf, M.D

    A solitary large nodule on the left cheek of a patient is biopsied and sent for histopathology.  Histology shows nodules of small to medium sized cells with condensed chromatin and slightly atypical, angulated nuclei. No epidermotropism is present. There are scattered intermixed eosinophils and plasma cells in the infiltrate. Immunohistochemistry shows that the atypical cells are positive for CD3, CD4, CD5, and PD-1. CD30 staining is negative. There is positive staining of CD20 B-cells but not of the atypical appearing cells. T-cell rearrangement studies are positive for a clonal proliferation.

    Histology showing a superficial and deep nodular infiltrate with no germinal centers present.

    High power field showing small lymphocytes with condensed chromatin and atypical nuclei. Admixed eosinophils and plasma cells are present.

    CD4 staining is strongly positive.

    CD20 staining is positive but not highlighting most of the infiltrate present.

    PD-1 staining highlighting the same CD4 positive cells.


    Which of the following is the best diagnosis?

    2014 Magro Lupus Panniculitis

    Multiple choice question

    Question prepared using materials provided by Dr. Cynthia M. Magro, M.D

    The patient, shown below, is presenting with tender, red plaques on the upper arm and thigh. Histology, shown below, shows the classic findings of a subcutaneous panniculitis-like T-cell lymphoma. There is a necrotizing, lymphocytic, lobular panniculitis. Vascular thrombosis and rimming of lymphocytes around the individual fat cells is present. There may be engulfment of RBCs by macrophages.


    Representative clinical photograph showing red to violaceous, bruise-like, indurated plaques on the thigh.


    Histology showing a necrotizing, lobular panniculitis with no overlying epidermal involvement.

    Immunohistochemistry of representative lesion.


    CCL5 staining highlighting the atypical lymphocytes and rimming of the adipocyte.

    Which of the following diseases below has to be considered in histological differential diagnosis of subcutaneous panniculitis-like T-cell lymphoma?

    2014 Kempf Lymphomatoid Papulosis

    Multiple choice question

    Question prepared using materials provided by Dr. Werner Kempf, M.D, Ph.D.

    The case shown below features a  wedge shaped infiltrate with central necrosis, scattered large anaplastic cells that stain positive for CD30, and admixed neutrophils and eosinophils that is consistent with a diagnosis of lymphomatoid papulosis. Type A histology (shown below) is the most common subtype.

    Low power histology showing a wedge shaped infiltrate with central epidermal necrosis.


    High power view showing large anaplastic cells.


    CD30 immunohistochemical stain highlighting the large atypical cells.

    All of the following descriptions are consistent with at at least one subtype (A - E) of lymphomatoid papulosis EXCEPT?

    2014 Guitart Folliculotropic Mycosis Fungoides

    Multiple choice question

    Question prepared using materials provided by Dr. Joan Guitart, M.D

    A patient presents with a patch of alopecia studded with large, crusted, pink plaques on the scalp, shown below. Shown below is a biopsy showing perifollicular infiltrate of atypical lymphocytes with infiltration of the follicular epidermis. Immunohistochemistry shows CD3+ cells with a CD4:CD8 ratio of 10:1 in the hair follicle. This is consistent with folliculotropic mycosis fungoides.

    Clinical photograph showing large, pink, plaques with some crusting and alopecia on the scalp.


    Histology showing an infiltrate composed of lymphocytes and eosinophils with involvement of the follicular epithelium.


    High power view showing involvement of the hair follicles with lymphocytes and scattered eosinophils. Immunohistochemistry showed a CD4:CD8 ratio of 10:1 in the hair follicle.

    Which of the following statements is true about this form of mycosis fungoides?

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