Non Hodgkin Lymphoma

What is it?

Almost 85% of NHLs are of B-cell origin; only 15% are derived from T/NK cells, and the small remainder stem from macrophages.

Represents a progressive clonal expansion of B cells or T cells and/or NK cells arising from an accumulation of lesions affecting proto-oncogenes or tumor suppressor genes, resulting in cell immortalization.

Subtypes NHL

Etiology

NHLs may result from:

chromosomal translocations

Infections

-Epstein-Barr virus (EBV)

-HIV

-Human T-cell leukemia virus type 1 (HTLV-1)

-Hepatitis C virus (HCV)

-Kaposi sarcoma–associated herpesvirus (KSHV)

-Helicobacter pylori

immunodeficiency states

-Congenital immunodeficiency states (eg, severe combined immunodeficiency disease [SCID], Wiskott-Aldrich syndrome),

-acquired immunodeficiency states (eg, AIDS),

Environmental factors

-chemicals (eg, pesticides, herbicides, solvents, organic -chemicals, wood preservatives, dusts, hair dye)

-chemotherapy

-radiation exposure.

maternal smoking during pregnancy

chronic inflammation

-Celiac disease

-Sjögren syndrome

-Hashimoto thyroiditis

History

Low-grade lymphomas

Peripheral adenopathy, painless

Primary extranodal involvement and B symptoms (ie, temperature >38°C, night sweats, weight loss >10% from baseline within 6 mo) are not common at presentation,

Bone marrow is frequently involved and may be associated with cytopenia or cytopenias.

Intermediate- and high-grade lymphomas

-adenopathy

-extranodal involvement;

GI) tract (including the Waldeyer ring), skin, bone marrow, sinuses, genitourinary (GU) tract, thyroid, and central nervous system (CNS). -B-symptoms are more common, occurring in approximately 30-40% of patients.

-Lymphoblastic lymphoma, a high-grade lymphoma, often manifests with an anterior superior mediastinal mass, superior vena cava (SVC) syndrome, and leptomeningeal disease with cranial nerve palsies.

Physical examination

Low grade:

-peripheral adenopathy

-splenomegaly

-hepatomegaly

Intermediate / high grade:

-Rapidly growing and bulky lymphadenopathy

-Splenomegaly

-Hepatomegaly

-Large abdominal mass : this usually occurs in Burkitt lymphoma

-Testicular mass

-Skin lesions

Staging

The Ann Arbor staging system.

Divide into 4 stages.

Prognosis

-International Prognostic Index (IPI)

-Follicular Lymphoma International Prognostic Index, (FLIPI)

The 5-year relative survival rate of patients with NHL is 71%.

Potential chemotherapy and other treatment-related complications:

-Nausea or vomiting

-Fatigue

-Neuropathy

-Dehydration after diarrhea or vomiting

-Cardiac toxicity from doxorubicin

-Catheter-related sepsis

-Catheter-related thrombosis

-Secondary malignancies

-Atherosclerosis

-Tumor lysis syndrome is characterized by hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, and renal failure

(Measures to prevent this complication include aggressive hydration, allopurinol administration, and urine alkalinization)

-Cytopenias

-AHA

-Bleeding secondary to thrombocytopenia, disseminated intravascular coagulation (DIC), or vascular invasion by the tumor

-Infection secondary to leukopenia, especially neutropenia

-Cardiac problems secondary to large pericardial effusion or arrhythmias secondary to cardiac metastases

-Respiratory problems secondary to pleural effusion and/or parenchymal lesions

-Superior vena cava (SVC) syndrome secondary to a large mediastinal tumor

-Spinal cord compression secondary to vertebral metastases

-Neurologic problems secondary to primary CNS lymphoma or lymphomatous meningitis

-GI obstruction, perforation, and bleeding in a patient with GI lymphoma (may also be caused by chemotherapy)

-Pain secondary to tumor invasion

-Leukocytosis (lymphocytosis) in leukemic phase of disease

Ddx:

-tumor or cancer, mets

-other hematologic malignancies

-TB

-hodgkin lymphoma

Workout

-FBC, look for cytopenias in advance disease, bone marrow infiltration

-LDH, high indicate poor prognosis, high tumor burden

-LFT, hepatic involvement

-Hypercalcemia, Acute form ATLL( adult T cell lymphoma leukemia)

-HIV serology

-CXR, mediastinal enlargement, pericardial effussion, mediastinal adenopathy

-CT scan, staging

-Gallium scan, detect initial sites of disease, reflect therapy response, and detect early recurrences.

-Obtain an ultrasound image of the opposite testis in male patients with a testicular primary lesion.

-MRI of the brain and spinal cord of patients who are suspected of having primary CNS lymphoma, lymphomatous meningitis, paraspinal lymphoma, or vertebral body involvement by lymphoma.

-biopsy

-Bone marrow aspirate and biopsy, staging

-Biopsy of extranodal sites, e.g. GI tract.

Lumbar puncture for cerebrospinal fluid (CSF) examination in selected cases.

Mx:

-Depend on subtypes

-Chemo, radio

-Surgical in GI or testicular involvement

Activity restriction:

-neutropenia – avoid contact with communicable disease

-thrombocytopenia- soft toothbrush, avoid razor

Advertisements

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s