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Hairy cell leukemia and DDx
B.Ambaselmaa MNUMS AUS 623
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
• 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
• 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.
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]
• 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
• 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
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)
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
• 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.
Differential Diagnoses
• Hodgkin Lymphoma
• Epstein-Barr Virus (EBV) Infectious
Mononucleosis (Mono)
• Thank you for your attention

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Hairy cell leukemia and d dx

  • 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.
  • 6.
  • 7.
  • 8.
  • 9.
  • 10.
  • 11. 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]
  • 12. • 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
  • 13. • 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
  • 14. 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)
  • 15. 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
  • 16. • 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.
  • 17. Differential Diagnoses • Hodgkin Lymphoma • Epstein-Barr Virus (EBV) Infectious Mononucleosis (Mono)
  • 18.
  • 19. • Thank you for your attention