Medical Nursing (III)
Subtopic:
Disease of lymph vessels
Lymphoedema/lymphatic dysfunction
Lymphoedema indicates swelling in body tissues resulting from a blockage in the lymphatic system, which prevents proper fluid drainage.
Lymphedema can also be defined as the accumulation of protein-rich fluid within the interstitial spaces of tissues due to a disruption in normal lymphatic flow.
When a lymph vessel becomes blocked, lymph fluid accumulates in the areas further down the system (distal parts). This accumulation can trigger mild inflammation within the affected tissues and lymph vessels themselves.
Lymphatic dysfunction signifies that the lymphatic system is not operating effectively or as it should.
Physiologic basis of lymphedema — Lymphedema develops when the amount of lymphatic fluid to be transported (lymphatic load) exceeds the lymphatic system’s capacity to move it. This imbalance leads to the build-up of filtered fluid within the tissue spaces (interstitium). Importantly, unlike general swelling (edematous states), the rate at which fluid filters out of capillaries is typically normal in individuals with lymphedema.
Causesof lymphoedema
Causes of Lymphoedema can be categorized as:
Primary: This type results from conditions present at birth (congenital) or inherited, which are linked to the abnormal development of lymphatic vessels.
Secondary: This form arises as a consequence of other medical conditions or treatments.
Congenital lymphatic obstruction (Milroy’s disease): This involves the failure of lymph vessels, particularly in the lower limbs, to develop properly. It is also known as hereditary or primary lymphoedema, highlighting its genetic basis.
Surgical removal of lymph vessel and lymph nodes: This is often done as part of cancer treatment, such as the removal of axillary lymph nodes during a mastectomy to prevent the spread of cancer. However, it can lead to lymphoedema in the affected arm by disrupting lymphatic drainage.
Tumours: Growths can exert pressure on lymph vessels, physically obstructing the flow of lymph and preventing proper tissue drainage.
Filariasis: This is a disease caused by thread-like worms (nematodes) that live in the tissues and are transmitted through mosquito bites. These worms can block lymphatic vessels.
Malignant metastasis of lymph node and lymph vessel: The spread of cancer cells to lymph nodes and vessels can also cause blockages and impair lymphatic function.
Signs and symptomsof lymphoedema
Typical signs and symptoms of lymphedema include:
The onset of lymphedema is often gradual (insidious). Initially, affected individuals might experience a dull, persistent ache (aching pain) in the affected area along with a sensation of heaviness or fullness in the limb. Over time, the skin can become dry and firm, and when pressed, it may show less of a temporary indentation (less pitting). The tissue may also feel dense and fibrous upon touch.
Two-thirds of lymphedema cases affect only one side of the body (unilateral), though which side is affected depends on the underlying cause. For instance, removing lymph nodes in the armpit (axillary node dissection) increases the risk of lymphedema in that same arm (ipsilateral), while removing lymph nodes in the pelvis (pelvic node dissection) raises the risk of swelling in both lower limbs (bilateral lower extremity edema).
At the beginning, swelling in the affected limb is usually described as “soft” and leaving a temporary pit when pressed (“pitting”). The presence and depth of pitting can vary and reflects the movement of excess water in the tissue in response to pressure. As lymphedema progresses, the pitting may disappear, indicating the development of fibrous tissue and fat deposits.
In individuals who have had lymph nodes removed and radiation therapy, lymphedema typically manifests as a slow, progressive swelling on the same side of the body as the node removal (ipsilateral). This could be an arm after axillary node dissection or a leg after inguinal (groin) node dissection. The swelling might first appear only in the upper part of the limb (proximal portion) or only in a part of the lower limb, such as the fingers or toes (digits).
For those with breast cancer, swelling can also occur over the breast on the affected side and/or the upper chest wall on that side. Other symptoms include a feeling of weight, tightness, aching, or discomfort in the limb, and a reduced ability to move the limb through its full range of motion.
Skin changes — As lymphedema worsens, the skin can become noticeably thicker (dermal thickening), a result of fibrous tissue buildup (cutaneous fibrosis). The skin on the affected limb can also become abnormally thick and scaly (hyperkeratotic), potentially leading to wart-like (verrucous) and small blister-like (vesicular) lesions.
Discomfort — A sensation of heaviness, tightness, aching, or general discomfort in the affected limb commonly accompanies the swelling.
Restricted range of motion — In the later stages of lymphedema, patients might find it difficult to move the affected limb fully due to the increased weight and tissue changes, which can limit their ability to perform everyday tasks (activities of daily living (ADLs)).
Summary of Clinical Features
Swelling in arms and legs is common.
The tissues of the neck and head can also be affected.
Swelling can lead to reduced movement (reduced mobility).
A sensation of weight or heaviness in the affected area.
Changes in the skin, such as discoloration.
The formation of fluid-filled blisters.
Fluid leaking from the skin surface.
Increased susceptibility to infections.
If the head and neck are affected, symptoms may include poor vision, ear pain, and nasal congestion.
Difficulty swallowing.
Trouble breathing and speaking.
Excessive saliva production (salivating).
Lymphangitis (inflammation of the lymph vessels) and cellulitis (bacterial skin infection) are potential complications. These may present as red streaks on the affected area, fever, chills, and itching.
Diagnosis and Investigation of lymphoedema
History: Gathering information about the patient’s condition. Key aspects to discuss include:
The age when the swelling first started (Age of onset).
Which part(s) of the body are affected (Area(s) of involvement).
Any other associated symptoms, such as pain.
Current medications – While no medication directly causes lymphedema, some can contribute to general swelling (e.g., NSAIDs), and some are not recommended for lymphedema treatment (e.g., diuretics).
How the symptoms have changed over time (Progression of symptoms).
Past medical history, including conditions linked to lymphedema, any travel history, infections, surgeries, or previous radiation therapy.
Whether anyone else in the family has experienced similar issues (Family history).
Physical Examination.
The physical exam should assess the blood vessel system (vascular system), the skin, the tissues beneath the skin (soft tissue), and involve feeling for enlarged or abnormal lymph nodes (palpation of the lymph nodes).If primary lymphedema is suspected, the examination should look for any physical features or birth defects (congenital anomalies) associated with inherited conditions. Examples include:
Short height (Short stature) seen in Turner Syndrome.
Birthmarks (Port wine stains) or benign blood vessel tumors (hemangiomas) seen in Klippel-Trenaunay-Weber Syndrome.
An unusually broad chest (Shield chest) seen in Turner Syndrome and Noonan Syndrome.
A positive Stemmer sign is a strong indicator of lymphedema. This is assessed by trying to pinch and lift the skin on the affected limb compared to the unaffected limb. A positive sign means it’s difficult or impossible to lift the skin, especially on the back of the fingers or toes. A positive Stemmer sign can be present at any stage of lymphedema. While a false negative result is possible, a false positive is rare.
Volume Measurement:
Opto electronic volume try: This technique uses infrared light to scan the limb and calculate its volume.
Circumferential measurements: A simple and cost-effective method involving measuring the circumference of the affected and unaffected limb at specific points. For the arm, measurements are typically taken at the finger joints (Metacarpal-phalangeal joints), the wrists, 10 centimeters below the elbow bone (lateral epicondyles), and 15 centimeters above the elbow bone. Similar measurements can be taken on the leg, head, neck, or trunk using consistent anatomical landmarks.
Water displacement: This method can detect very small changes in volume (less than 1 percent). For limb lymphedema, a volume difference of 200 mL or more between the affected and unaffected limbs is generally considered indicative of lymphedema.
Bioimpedance spectroscopy (BIS): This is a reliable and accurate tool for measuring fluid volume by analyzing the resistance to a small electrical current passed through the body.
Imaging
Computed tomography (CT) scan: Can show fluid buildup within the soft tissues.
MRI: Magnetic resonance imaging is also used to evaluate lymphedema.
Lymphoscintigraphy: This technique involves injecting a radioactive tracer to track the flow of fluid from the skin to the lymph nodes, particularly in the arms and legs.
X-ray of lymphatic system: This involves injecting a dye between the toes or in the groin to visualize the lymphatic vessels.
Genetic testing: For individuals diagnosed with primary lymphedema or suspected of having late-onset lymphedema (lymphedema tarda), referral to a genetic specialist or counselor is recommended to assess family history and suggest further testing.
Blood smear: To look for the presence of filarial worms in cases where filariasis is suspected.
Stages of Lymphedema.
Staging helps to classify the severity of lymphedema using criteria from the International Society of Lymphology. The staging considers how “soft” or “firm” the limb is (reflecting the amount of fibrous tissue) and how the swelling responds to elevation:
Stage 0: This is a subclinical or hidden stage where there is impaired lymphatic transport, but visible swelling is not present. Most individuals in this stage don’t have symptoms, although some may report a feeling of heaviness in the limb. Stage 0 can last for months or even years before progressing to overt lymphedema (stages I to III).
Stage I: Fluid accumulation is present, but the swelling reduces when the limb is elevated for 24 hours. The swelling feels soft and may leave a pit when pressed. There are no signs of fibrous tissue buildup in the skin. This stage is sometimes referred to as reversible edema.
Stage II: The swelling does not fully resolve with 24 hours of limb elevation alone, indicating the development of fibrous tissue in the skin (dermal fibrosis). As the fibrosis progresses, the limb may no longer show pitting when pressed. This stage is sometimes called spontaneously irreversible lymphedema.
Stage III: This is the most severe stage, characterized by lymphostatic elephantiasis. The limb is significantly swollen, feels firm, and does not pit on examination. The skin shows noticeable changes (trophic skin changes) such as fat deposits, thickened skin (acanthosis), and wart-like growths
Classification of lymphedema
Several classification systems are utilized to categorize lymphedema, including those from the American Physical Therapy Association (APTA) and the National Cancer Institute’s Common Terminology Criteria for Adverse Events (CTCAE). The APTA system, which relies on limb circumference measurements, is often preferred. Both methods are described below.
The APTA classification uses the difference in girth between the affected and unaffected limb as a measurement. The largest difference in circumference determines the category of lymphedema (See ‘Circumferential measurements’ previously described):
Mild lymphedema — The maximum difference in girth is less than 3 centimeters.
Moderate lymphedema — The difference in girth ranges from 3 to 5 centimeters.
Severe lymphedema — The difference in girth is greater than 5 centimeters.
The National Cancer Institute Common Terminology Criteria for Adverse Events (CTCAE) classifies lymphedema based on observable signs during an examination and the presence of any limitations in function:
Grade 1 — Minimal tissue thickening or a slight change in skin color.
Grade 2 — Noticeable skin discoloration; the skin may feel tough or leathery; small, wart-like growths (papillary formation) may be present; daily activities requiring tools or equipment are difficult to perform (limiting instrumental activities of daily living – ADL).
Grade 3 — Severe symptoms that significantly restrict the ability to perform basic self-care activities (limiting self-care ADL).
Management of lymphoedema
Compression of the affected limb to promote lymph drainage: Applying external pressure helps to move fluid out of the swollen area.
Wrapping the affected limb in elastic bandage to maintain continuous pressure on swollen limb to reduce the size and improve mobility: Bandaging provides sustained support and aids in reducing swelling.
Use of compression garments (special designed socks), stockings, or sleeves that have a comfortable fit over the swollen limb (manual lymphatic drainage/lymphatic drainage massage): Specialized garments provide ongoing pressure, and massage techniques can further encourage lymphatic flow.
Exercise can help fluid move from the vessels and reduce on swelling such as knee bending, wrist rotation, swimming, walking, etc for 20 to 30 minutes daily: Specific exercises can stimulate lymphatic circulation.
Routine skin care: Keeping the skin clean and moisturized is crucial to prevent infections.
Liposuction in advanced stages of lymphedema when other options fail: Surgical removal of fat may be considered in severe cases.
Anti biotic are prescribed prevent spread of infections: Medications to treat or prevent bacterial infections.
Treat secondary fever with analgesics: Pain relievers can help manage fever.
Ensure good diet to improve immunity: Proper nutrition supports overall health and the body’s defense system.
Lymphatic venous anastomosis: A surgical procedure to connect lymphatic vessels to small veins to improve drainage.
Massaging: Gentle massage techniques can encourage lymph fluid movement.
Elevation: Raising the affected limb can help reduce swelling by using gravity.
Apply pressure on the affected areas: Similar to bandaging, this aids in fluid movement.
Complete decongestive therapy: A comprehensive treatment approach combining multiple techniques like massage, bandaging, exercise, and skin care.
Surgery due to obstruction: In some cases, surgery may be needed to bypass or remove blockages.
Lymphangitis
Lymphangitis describes the inflammation of lymph vessels, typically caused by a bacterial infection.
When microorganisms enter the lymph, draining an infected area, they can spread along the walls of the lymph vessels. This spread may be contained within the first lymph node encountered or may continue, affecting the entire lymphatic drainage network and even entering the bloodstream.
Potential infectious agents include:
bacteria
mycobacteria
viruses
Fungi
parasites
Lymphangitis most often develops after microorganisms are introduced into the lymphatic vessels through a break in the skin (cutaneous inoculation), such as a wound, or as a complication of an existing infection further down the limb (distal infection).
Pathophysiology of Lymphangitis
The primary function of the lymphatic system is to collect excess fluid and proteins from tissues and spaces outside of blood vessels. Lymphatic endothelial cells lack a basement membrane, giving lymphatic channels a unique ability to absorb proteins that are too large for venules to absorb.
Lymphatic channels are located in the deeper layer of the skin (deep dermis) and the tissues beneath the skin (subdermal tissues), running parallel to veins. They contain a series of valves to ensure that lymph flows in only one direction. Lymph drains through incoming (afferent) lymphatic vessels to regional lymph nodes and then through outgoing (efferent) lymphatic vessels to the cisterna chyli (a sac-like structure) and the thoracic duct, eventually emptying into the subclavian vein and the general blood circulation.
Lymphangitis occurs when microorganisms enter the lymphatic vessels, either directly through a skin wound or abrasion or as a consequence of an infection located further away. These organisms then travel towards the regional lymph nodes.
Pathophysiology of Edema
The circulation of lymph is a complex process. All body tissues are surrounded by interstitial fluid. When there is too much of this fluid, it accumulates, leading to swelling (oedema).
Therefore, edema occurs due to:
Excessive production of interstitial fluid: This can happen due to increased leakiness of capillaries during inflammation, reduced protein levels in the blood (low colloid osmotic pressure in hypoproteinemia), or high pressure in the veins (high venous pressure in thrombosis).
Inadequate removal/ transport of interstitial fluid by lymphatic system: When the lymphatic system cannot effectively drain fluid, it accumulates, a condition known as lymphoedema.
Normally, about 2-4 liters of interstitial fluid are filtered out of capillaries each day and returned to the bloodstream by the lymphatic system. The movement of fluid across capillary walls depends on the balance between hydrostatic pressure (pushing fluid out) and oncotic pressure (pulling fluid in), with a net outward flow at the arterial end of capillaries and a net inward flow at the venular end.
Causes
Swelling in the limbs (Extremity oedema) can result from conditions like right-sided heart failure, constrictive pericarditis (inflammation of the sac around the heart), kidney diseases, liver scarring (cirrhosis), and low protein levels in the blood (hypoproteinemia).
Other causes include:
Acute or chronic obstruction in the venous system
Abnormalities in lymphatic system
Allergic disorders
Clinical Manifestation of Lymphangitis
Lymphangitis is characterized by red streaks on the skin, accompanied by pain and rapid spreading, or by the appearance of bumps or nodules along the path of the lymphatic vessels.
Acute lymphangitis – Often occurs following a skin break with infection at a site further down the limb, such as a fungal infection between the toes (interdigital dermatophyte infection) or a bacterial skin infection (cellulitis) of the lower leg. This may involve red, tender streaks extending upwards, along with inflammation of the regional lymph nodes (lymphadenitis). Systemic symptoms like fever may also be present.
In individuals with a healthy immune system, the most common cause is Streptococcus pyogenes. Staphylococcus aureus can also be a cause. In those with weakened immune systems, gram-negative bacteria are important causes of lymphangitis following lower leg cellulitis.
Pasteurella multocida, from dog and other animal bites, can lead to a localized infection with accompanying lymphangitis. It occurs in up to 30 percent of infections with Erysipelothrix, a disease contracted by people in contact with fish and certain animals. Cutaneous anthrax can present with significant swelling, regional lymph node enlargement, and lymphangitis.
Lymphangitis associated with Rickettsial infections has been described with the organisms R. sibirica mongolotimonae and R. africae (causing African tick bite fever). The presence of a scab at the site of the bite (inoculation eschar) suggests a tick-borne infection.
Nodular lymphangitis
Nodular lymphangitis (also known as sporotrichoid lymphangitis, sporotrichoid spread, or lymphocutaneous syndrome) appears as painful or painless bumps under the skin along the lymphatic vessels.
Sporotrichosis, a fungal infection, can occur after injury from gardening or contact with thorns or wood splinters. It can have an incubation period of up to three months and can present as a skin infection or as a lymphocutaneous form accompanied by lymphangitis; painless ulcers may also be seen. Lesions on the arms are the most common. These lesions can ulcerate, and the regional lymph nodes may become enlarged. The time between exposure and the onset of nodular lymphangitis can be long, and the presentation may be slow-developing with few or no general symptoms.
Causes of nodular lymphangitis include: Sporothrix schenckii (a fungus), Nocardia bacteria (most often N. brasiliensis), Mycobacterium marinum, Leishmania parasites, Francisella tularensis bacteria (causing tularemia), and certain systemic fungal infections.
M. marinum can cause “fish tank granuloma,” which can occur after a hand injury while cleaning a fish tank. It has an incubation period of up to eight weeks. Infections from fast-growing mycobacteria can also occur after a thorn or splinter injury. Other mycobacterial causes of lymphangitis include M. kansasii, M. chelonae, and M. fortuitum.
Nocardia infections may appear as skin, subcutaneous, or lymphocutaneous issues following an injury. While it may look similar to an acute staph or strep infection, it usually develops more slowly.
Cutaneous leishmaniasis can appear as nodules under the skin with lymphangitis or lymph node inflammation up to 24 weeks after exposure. This is more common with leishmaniasis caused by L. braziliensis or L. mexicana (found in the Americas) than with L. major or L. tropica (found in the Old World).
Nodular lymphangitis is a rare sign of infection with Francisella tularensis and Burkholderia pseudomallei. The initial skin lesion of a F. tularensis infection may be a small bump, an ulcer, or a sore with a dark center (eschar).
Systemic fungal infections like coccidioidomycosis, blastomycosis, and histoplasmosis can sometimes present with nodular lymphangitis.
Filarial lymphangitis — The presence of filarial parasites within the lymphatic channels causes inflammation, leading to widening, thickening, and twisting of the lymphatic vessels, along with malfunctioning valves. This often progresses in a backward direction, spreading away from the regional lymph nodes where the adult parasites live. It can also occur due to inflammation caused by dying parasites.
Wuchereria bancrofti, Brugia malayi, and Brugia timori are the parasites that cause lymphangitis due to lymphatic filariasis.
Diagnosis of Lymphangitis.
Diagnosis involves assessing the clinical signs and symptoms, along with laboratory analysis of clinical samples.
Microbiological investigations
Swab, aspirate, and biopsy of the primary infection site, nodules, or distal ulcers for examination under a microscope (including Gram stain, fungal stains, and acid-fast stains) and for culturing bacteria, fungi, and mycobacteria.
Serology (blood tests) for specific infections (e.g., F. tularensis, Histoplasma).
Blood film to look for parasites (e.g., filaria).
Imaging — Lymphangiography (using dye injected into the lymphatics) and lymphoscintigraphy (using an injection of radioactive material under the skin at the affected limb) have been used to evaluate lymphedema and lymphatic obstruction. This may be helpful if surgery is being considered for lymphedema.
Treatment of Lymphangitis
Some cases of nodular lymphangitis require surgical removal of damaged tissue (debridement). Surgery may also be appropriate for lymphedema with significant lymphatic obstruction. While awaiting diagnostic results, treatment with antibiotics that are effective against common skin bacteria may be started.
DISEASES OF LYMPH NODES
Lymphadenitis/adenitis
Lymphadenitis refers to the inflammation of lymph nodes, usually due to a bacterial infection spreading from infected lymph vessels.
The lymph nodes become enlarged, tender, and filled with blood and inflammatory substances (chemotaxins).
If the body’s defenses (phagocytes and antibodies) are overwhelmed, an abscess (collection of pus) may form within the node. Nearby tissues may become involved, and infected material can be transported to other lymph nodes and the bloodstream.
Lymphadenitis can be acute (sudden onset) or chronic (long-lasting). In children, the lymph nodes in the neck (cervical lymph nodes) are most commonly affected.
Causesof acute lymphadenititis
Measles
Typhoid fever
Cat-scratch fever
Wound infections
Skin infections
Causesof chronic lymphadenititis
Tuberculosis (TB)
Syphilis
Unresolved acute infections
Signs and symptomsof lymphadenitis
Tenderness of the affected lymph nodes.
Redness of the skin around the affected nodes.
Fever in severe cases.
Lymphadenopathy
This refers to the enlargement of lymph nodes.
Lymphadenopathy can be localized (affecting nodes in one area) or generalized (affecting nodes in multiple areas).
Causes of generalized lymphadenopathy include:
Infections like TB, HIV, and syphilis.
Acute and chronic lymphoid leukemia (cancers of white blood cells).
Lymphoma like Hodgkin’s lymphoma and Non-Hodgkin’s lymphoma (cancers of the lymphatic system).
Sarcoidosis (an inflammatory disease).
Causes of localized lymphadenopathy include:
Localized infections, for example, a scalp infection leading to cervical lymph node enlargement, an infection of the arm leading to axillary lymph node enlargement, an infection of the leg leading to inguinal lymph node enlargement, and an infection of the throat or tonsils leading to mandibular lymph node enlargement.
Carcinoma (cancer) that has spread to the lymphatics, causing regional lymphadenopathy, e.g., stomach cancer leading to supraclavicular lymphadenopathy, breast cancer leading to axillary lymphadenopathy.
DISEASES OF THE SPLEEN
Splenomegaly/ hypersplenism
Splenomegaly refers to the enlargement of the spleen beyond its normal size (approximately 12 x 7 cm).
The enlarged spleen may not be felt during a physical exam (non palpable) but can be detected on imaging scans. It usually becomes palpable only when it is more than two and a half times its normal size.
The spleen can become infected by microorganisms carried in the blood or through the local spread of infection. Enlargement of the spleen is associated with several problems, including:
Premature destruction of red blood cells.
Sequestration (trapping) of red blood cells, white blood cells, and platelets (pancytopenia).
Increased vulnerability to traumatic rupture.
The bone marrow may respond by increasing its production of blood cells (hyperplasia of bone marrow) as a compensatory mechanism.
Causes of splenomegaly
Bacterial infections like endocarditis (infection of the heart lining), tuberculosis, septicemia (blood poisoning), brucellosis, syphilis, and typhoid fever.
Viral infections like hepatitis and AIDS.
Protozoal infections like malaria, leishmaniasis, and trypanosomiasis.
Fungal infections like histoplasmosis.
Inflammatory disorders like SLE (lupus), rheumatoid arthritis, and sarcoidosis.
Congestion due to portal hypertension (high blood pressure in the portal vein), hepatic vein thrombosis (blood clot in the hepatic vein), chronic congestive heart failure (CCF), and pericardial effusion (fluid around the heart).
Hemolytic disorders like sickle cell anemia and spherocytosis (conditions where red blood cells are destroyed prematurely).
Infiltrative diseases of the spleen like leukemia and lymphomas (cancers affecting blood cells and lymphatic tissue).
Iron deficiency anemia and megaloblastic anemia (types of anemia).
Idiopathic (unknown cause).
Signs and symptoms of splenomegaly
Palpable spleen (can be felt during a physical exam).
Fever.
Jaundice (yellowing of the skin and eyes).
Other signs of the underlying disease causing the splenomegaly.
Management of splenomegaly
Investigations help in identifying the cause, such as blood smear, CBC, radiological imaging, and culture and biopsy.
Treat the underlying cause accordingly.
In severe, persistent hypersplenism (overactive spleen), splenectomy (surgical removal of the spleen) may be performed.
Hyposplenism
This refers to the reduced or absent function of the spleen.
It can be due to:
Trauma to the spleen.
Surgical removal of the spleen.
Auto splenectomy, as seen in sickle cell disease due to tissue damage from blocked blood vessels (ischemic atrophy).
Splenic atrophy in celiac disease.
Signs of hypoplenism
Recurrent bacterial infections, particularly from organisms like streptococci, E. coli, Haemophilus influenzae, and Neisseria meningitides.
Severe septicemia (blood poisoning).
Disseminated intravascular coagulation (DIC) (a condition where blood clots form throughout the body).
Multiorgan failure.
Abnormalities in red blood cells.