Hairy cell leukemia and d dx

from harrison and other resources

1. Hairy cell leukemia and DDx B.Ambaselmaa MNUMS AUS 623

2. Hairy cell leukemia Hairy cell leukemia is a rare disease that presents predominantly in older man. Typical presentation involves pancytopenia, although occasional patients have a leukemic presentation . Splenomegaly is usual. The malignant cells

3. appear to have “hairy” projections on light and electron microscopy and show a characteristic staining pattern with tartrate-resistant acid phosphatase. Bone marrow is typically not able to be aspirated, and biopsy shows a pattern of fibrosis with diffuse infiltration by the malignant cells. Patients with this disorder are prone to

4. unusual infections, including infection by Mycobacterium avium intracellulare , and to vasculitic syndromes. Hairy cell leukemia is responsive to chemotherapy with interferon γ, pentostatin , or cladribine , with the latter being the usually preferred treatment. Clinical complete remissions with cladribine occur in most patients, and longterm disease-free survival is frequent.

5. Hodgkin Lymphoma Differential Diagnoses Diagnostic Considerations Other conditions to consider in the differential diagnosis of Hodgkin lymphoma include the following: Any disease presenting with lymphadenopathy and constitutional symptoms Infection with human immunodeficiency virus (HIV) Hypersensitivity reaction Other solid tumors Non-Hodgkin lymphoma , particularly  diffuse large B cell lymphoma  and  anaplastic large cell lymphoma , both of which may have CD30 expression [34]

6. Because Hodgkin lymphoma is considered a curable malignancy and the differential diagnosis is broad, medicolegal problems may arise from failure to diagnose the disease in a timely manner, possibly due to the following factors: The misinterpretation of B symptoms A lack of follow-up for abnormal chest radiographs or physical examination findings A missed pathologic diagnosis because a needle biopsy was obtained rather than an excisional lymph node biopsy

7. Occasionally, Hodgkin lymphoma can present as hemophagocytic syndrome ( hemophagocytic lymphohistiocytosis ).  [35]  The hemophagocytic syndrome may be more prevalent in patients with Epstein-Barr virus (EBV) antigen expression and has the following characteristics: Pancytopenia Fever Hepatosplenomegaly with liver function test abnormalities Elevated serum levels of ferritin and triglycerides Phagocytosis of hematopoietic lineage cells by benign macrophages

8. Differential Diagnoses Cytomegalovirus (CMV) Epstein-Barr Virus (EBV) Infectious Mononucleosis (Mono) Non-Hodgkin Lymphoma Physical Medicine and Rehabilitation for Systemic Lupus Erythematosus Sarcoidosis Serum Sickness Small Cell Lung Cancer Syphilis Toxoplasmosis Tuberculosis (TB)

9. Non-Hodgkin Lymphoma Differential Diagnoses Diagnostic Considerations A significant number of medical disorders can produce local or generalized lymph node enlargement. Thus, the diagnosis of non-Hodgkin lymphoma (NHL) relies on pathological confirmation following appropriate tissue biopsy. The following are some of the conditions that can result in clinical manifestations similar to those observed in lymphoma patients: Solid tumor malignancies: metastatic disease to lymph nodes secondary to carcinoma, melanoma, or sarcoma

10. Other hematologic malignancies or lymphoproliferative disorders: granulocytic sarcoma, multicentric Castleman disease Benign lymph node infiltration or reactive follicular hyperplasia secondary to infection ( eg , tuberculosis; other bacterial, fungal, and, rarely, viral infections), and collagen-vascular diseases Hodgkin lymphoma, which requires a different treatment strategy than NHL If the diagnosis of NHL is doubtful, acquire a second or third opinion from an expert hematopathologist before instituting therapy. Flow cytometry and cytogenetics should also be performed and can be especially helpful in difficult situations.

11. Differential Diagnoses Hodgkin Lymphoma Epstein-Barr Virus (EBV) Infectious Mononucleosis (Mono)

12. Thank you for your attention