Hematologic Malignancy

1

What are the broad classes of hematologic malignancies?

Hematologic malignancies include leukemia, lymphomas, and multiple myeloma. Leukemias are either acute or chronic and are classified based on the myeloid or lymphoid lineage. Lymphomas are further categorized as B-cell (Hodgkin’s and non-Hodgkin’s) or T-cell neoplasms.

2

What are the head and neck manifestations of hematologic malignancies?

This can be broadly divided into nodal and extranodal manifestations.

• Nodal: Cervical lymphadenopathy is a common presentation of lymphomas and is also observed in chronic lymphocytic leukemia (CLL). Certain lymphomas present with Waldeyer’s ring involvement (clinically apparent as adenotonsillar hypertrophy).

• Extranodal: Involvement of lymphoid tissues in the salivary glands, thyroid, and paranasal sinuses may present as masses in these regions. Endemic Burkitt’s lymphoma has a distinct propensity to present as a mass of facial bones. Extramedullary plasmacytomas can originate in the sinonasal tissues. Mediastinal lymphadenopathy could cause compression of the trachea or superior vena cava with findings of dyspnea, airway compression while lying supine, or facial edema. Other presentations are summarized in Table 21.1 .

  Table 21.1

  ENT Manifestations of Hematologic Malignancies

  I. Nodal	Lymphadenopathy Involvement of Waldeyer’s ring
  II. Extranodal	Nasal obstruction Paranasal sinus involvement Facial bone erosion Thyroid infiltration Salivary gland involvement Airway obstruction (direct or from tracheal compression)
  III. Vascular	Superior vena cava (SVC) syndrome
  IV. Neurologic	Cranial nerve palsy (if CNS involvement) Mental nerve involvement Recurrent laryngeal nerve palsy
  VI. Cytopenias	Anemia: mucosal pallor Thrombocytopenia: epistaxis, mucosal petechiae, purpura Neutropenia: sore throat

3

In a patient with cervical lymphadenopathy, what features should alert the clinician to the possibility of lymphoma?

Generalized symptoms along with lymphadenopathy raise the suspicion of malignancy. (1) Unexplained fevers (temperature > 38°C during previous month), (2) unintentional weight loss (>10% of body weight during the previous 6 months), and (3) drenching night sweats during the previous month are the classically designated “B” symptoms and portend a poor prognosis. Although nonspecific, approximately 25% of patients with Hodgkin’s lymphoma and up to 40% of patients with non-Hodgkin’s lymphoma present with “B” symptoms. Fatigue is an additional symptom. Hodgkin’s lymphoma is uniquely associated with pruritus and pain after alcohol consumption.

4

What are the physical exam characteristics of lymphadenopathy in lymphomas?

Lymphoma-involved nodes are nontender and could be matted and fixed to underlying structures; one of the physical examination findings is multiple, bulky lymph nodes in several locations. It is important to examine the supraclavicular, axillary, and inguinal areas for lymphadenopathy.

5

What is lethal midline granuloma?

This term is used to describe an aggressive form of extranodal natural killer/T-cell lymphoma mediated by Epstein-Barr virus infection. It is common in East Asia and Latin America. Patients present with destructive masses involving the nasal cavity, sinuses, or palate, sometimes with extension into the upper airway and Waldeyer’s ring. The tumors are fast growing and may cause airway obstruction. Biopsy of these lesions usually reveals extensive necrosis with lymphomatous infiltration and vascular invasion. Localized disease is responsive to concurrent chemoradiotherapy, but advanced-stage disease is rapidly fatal despite treatment.