Lymphoid
Mature B-cell neoplasms
B-cell prolymphocytic leukemia
Splenic marginal zone lymphoma
Hairy cell leukemia
Nodal marginal zone B-cell lymphoma
Mediastinal large B-cell lymphoma
Intravascular large B-cell lymphoma
Primary effusion lymphoma
Lymphomatoid granulomatosis
Mature T-cell and natural killer (NK) cell neoplasms
T-cell prolymphocytic leukemia
T-cell large granular lymphocytic leukemia
Aggressive NK cell leukemia
Extranodal NK/T-cell lymphoma, nasal type
Enteropathy-type T-cell lymphoma
Hepatosplenic T-cell lymphoma
Subcutaneous panniculitis-like T-cell lymphoma
Blastic NK cell lymphoma
Primary cutaneous CD30+ T-cell lymphoma
Angioimmunoblastic T-cell lymphoma
Myeloid
WHO Classification of Myeloid Malignancies
Histiocytic and Dendritic Cell Neoplasms
Histiocytic sarcoma
Langerhans cell histiocytosis
Langerhans cells are specialized dendritic cells found predominantly in the skin.
- Derived from bone marrow monocytes
- Present antigen to naïve T cells
Characteristic Birbeck (tennis racket) granules are seen on electron microscopy; cells are CD1a+ and S100+ by immunohistochemistry.
Clinical findings
- Lytic bone lesions
- eg, skull, jaw, femur
- May be asymptomatic or associated with local pain and an overlying tender mass.
- Although benign, they can be locally destructive and are typically treated with curettage or chemotherapy.
- Skin lesions
- Purplish papules, eczematous rash
- Lymphadenopathy, hepatosplenomegaly
- Pulmonary cysts/nodules
- Central diabetes insipidus
????, ???, ????? ???, ????? ??? ??, ? · ?? ??? ????.
??? ??? erythematous papules, nodules, and/or scaling plaques.
- Letterer-Siffe disease
- Malignant proliferation of Langerhans cells
- Skin rash and cystic skeletal defects in an infant(< 2 years old).
- Multiple organs may be involved; rapidly fatal
- Eosinophilic granuloma
- Benign proliferation of Langerhans cells in bone
- Pathologic fracture in an adolescent; skin is not involved.
- Biopsy shows Langerhans cells with mixed inflammatory cells, including numerous eosinophils.
- Hand-Schüller-Christian disease
- Malignant proliferation of Langerhans cells
- Scalp rash, lytic skull defects, diabetes insipidus, and exophthalmos in a child.
Treatment
- Solitary bone lesions in a low-risk anatomical region
- Asymptomatic lesion: a wait-and-watch approach may be appropriate
- Symptomatic lesion: curettage and/or excision (as a part of biopsy)
- Bone lesion in a high-risk anatomical region, multifocal bone involvement, or systemic manifestation
- Chemotherapy (Vinblastine and prednisone)
- Desmopressin for diabetes insipidus
Leave a Reply