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Splenomegaly: the patient with an enlarged spleen

  1. Gastroepato
  2. Gastroenterology
  3. Splenomegaly
  4. Painful abdominal points
  5. Portal hypertension circles

notes by dr Claudio Italiano 

The spleen functions as a kind of large lymph node, since it is an organ responsible for filtering blood from the pathogenic noxae, as well as having a blood reservoir function in case of any bleeding. For these reasons, the condition defined as splenomegaly does not represent a disease in itself, but the spy of many infectious diseases, or traumas, pathologies of the liver (see cirrhosis of the liver and portal hypertension, or autoimmune processes, or neoplasms in general, or leukemia and lymphomas Since the spleen functions as a large body lymph node, splenomegaly, in general, can mean, as a symptom, a reactive hyperplasia, that is, the physiological response to a fact infection or inflammation), cell proliferation or infiltration neoplastic, extramedullary hematopoiesis, proliferation of cells with macrophage function and congestion of the portal circle, of which the splenic vein is a tributary. From an objective point of view, the spleen is hardly palpable, except underneath the costal arch, which the experienced doctor can do, and can be detected by gently palpating beneath the left shoulder margin. When this maneuver becomes difficult, for examples in obese people, then the CT scan or scintigraphy or a simple ultrasound scan are sufficient investigations for diagnostic purposes, to identify a splenomegaly.

Urgent interventions

Unfortunately, it may happen to the emergency room physician, to come across an accident patient; for example, who suffered a motorcycle accident and reported a closed trauma of the abdomen with ruptured spleen, especially if the spleen is enlarged. In these cases, if these signs are found:
- shock state
- acute anemia
upper left quadrant pain with abdominal resistance (see pain, upper abdominal pain left, surgical abdominal pain)
then it is necessary as soon as possible to cannulate a vein, to make a withdrawal, to check the vital parameters, such as breathing, cardiac activity, volemia (see resuscitation) and to start in the operating room, without delaying or palpating because of the risk of aggravating the internal bleeding.

Causes of Splenomegaly

Increase of function

- Removal of defective red blood cells: in the course of spherocytosis, thalassemia, hemoglobinopathies, autoimmune hemolitic anemia
- Response to infections (viral, bacterial, mycotic, parasitic): first think of leishmaniasis (!), Brucellosis, mononucleosis, malaria, AIDS, viral hepatitis, subacute bacterial endocarditis, bacterial septicemia, abdominal typhus, tuberculosis, syphilis, splenic abscess, typhoid fever, sarcoidosis, amyloidosis
- Vascular hypereflex: Hepatic vein obstruction, Port vein obstruction, Budd-Chiari syndrome, Splenic vein obstruction
- Immunoregulation disorders: Rheumatoid arthritis, Systemic lupus erythematosus, Serum disease,
- Extramedullary Hematopoiesis: Myelofibrosis, Infiltration of Bone by Tumors or Leukemia, Bone Damage from Radiation or Toxins
- Benign and malignant infiltrations: leukemia (acute, chronic, lymphoid and myeloid), lymphomas (Hodgkin and non-Hodgkin), myeloproliferative diseases, reticulosarcoma, metastatic tumors (commonly melanoma), histiocytosis X, hemangioma, lymphangioma

Diagnostic imaging

Ultrasonography: first level examination, immediately after the patient's visit, plays a role of confirmation in the presence of splenomegaly, allowing the differential diagnosis with palpable masses of other nature; normal adult max values: 12 cm woman, 13 cm man) and the Splenic Volumetric Index, for splenic dimensional monitoring other methods can be used, including: Splenic Weight Calculus, multiplying the three main diameters (longitudinal x anteroposterior x cross) for 0.43, which expresses the specific weight of the spleen. The normal value of the splenic weight thus calculated is less than 200 gr. In the case of splenomegaly, this can be distinguished in three degrees: mild (<500 g), medium (<2 kg), severe (> 2 kg).
Calculation of the area of ​​the maximum section; this is measured by surrounding the perimeter of the spleen with the ultrasound marker, in a longitudinal ultrasound section passing through the ilo; the area calculation is then performed through a specific keyboard command, implemented in all current production ultrasounds (normal values ​​<47 cmq).Tac addome: indagine di secondo livello, fornisce ulteriori informazioni

Bone marrow examination: shows hyperplasia of the medullary precursors of the cell line which appears to be reduced in the circulating blood and which manifests as peripheral cytopenia; lymphocyte infiltration in a lymphoproliferative disease, hyperplasia of myeloid elements in myeloproliferative disorders, increase of cellular blasts in acute leukemias, fibrosis in myeloid metaplasia, infiltration of a tissue that is colored with periodic Schiff's acid in amyloidosis and macrophages full of lipids in Gaucher's disease and other tesaurismosis.
Scintigraphy: scintigraphy with labeled technetium colloid is a feasible and non-invasive technique by which a mass is identified in the left hypochondrium as a spleen and an intrasplenic disease can be identified.

Anamnesis and objective examination

If splenomegaly is observed during a routine objective examination, it is necessary to reflect and begin to detect associated signs and symptoms.
Has the patient been asthenic and tired?
Is he anemic? Has there been a surge of white blood cells and relative to what underpolation? Leukocytes, lymphocytes, monocytes?
If the monocytes have increased and there has been fever and sore throat, can we think of the disease of the kiss, has the patient had a history of liver disease, jaundice, ascites? Then it is necessary to think about portal hypertension with venous stasis. Is the patient thalassaemic, has hemolytic anemias? Then we think that the spleen functions as a sort of blood filter and that collects aged red blood cells (reticulo-histiocytic system). Are there palpable lymph node stations? Are there lymphadenopathy? We think of lymphomas, we think of neoplasia. Is the patient a hunter? He loves dogs, he kisses them, licks them if he hugs them with enthusiasm? He lost weight, was he disappointed? We also think about infectious facts, we think of leishmaniasis. We even think of malaria, especially if it has recurrent fevers, which come and go intermittently! Do you have renal insufficiency, oliguria? Is it decompensated? There is lymphatic stasis, is there a stasis of a circle?

Pathologies and splenomegaly

Amyloidosis

In this pathology a marked splenomegaly can develop, deriving from an excessive deposit of protein material in the milaza, amorphous and whitish material. The symptoms are vague, the diagnosis in these cases is complicated and can be done only by performing a biopsy. The patient will have the most disparate signs ranging from renal failure to oliguria and anuria, to heart failure, to dyspnoea. At the gastrointestinal level you may have a strange diarrhea with malabsorption or constipation or hardening of the tongue and dysarthria.

Brucellosis

The patient ate fresh cheese, bought in the mountains by the shepherd, that genuine cheese, with whey! In severe cases of this rare infec- tion, splenomegaly is one of the main signs. Usually, brucellosis has an insidious onset, characterized by asthenia and elevated fever, headache, lumbago, anorexia and arthralgias. In later stages, hepatomegaly, lymphadenopathy, weight loss and painfulness can be observed at the pressure of the vertebrae or peripheral nerves.

Cirrhosis of the liver

Approximately one third of patients with advanced liver cirrhosis suffer from moderate or severe splenomegaly. Other late signs include jaundice, hepatomegaly, lower limb edema, hematemesis and ascites. There are also frequent signs of hepatic encephalopathy, such as asterixis, fetor hepaticus, clumsy speech, a reduction in the level of consciousness that can progress to coma. In addition to jaundice, skin effects may include severe pruritus, poor tissue turgidity, stellar angioma, palmar erythema, pallor, and signs of bleeding diathesis. At the endocrinological level, menstrual irregularities or atrophy can occur, testicular with signs of gynecomastia and loss of representation of hair on the chest and armpits. Patients may also have fever and pain in the upper right abdominal quadrant which is aggravated when seated.

Endocarditis

(infectious subacute). This infection usually causes the appearance of an enlargement of the spleen, however not painful. The classic signs, however, are. sudden modification of a breath or the appearance of a breath in the presence of fever. Other signs include anorexia, weakness, night sweats, tachycardia, weight loss, arthralgia, hematuria, petechial haemorrhages, still the patient can complain of chest pain, pain in the abdomen, limbs, paralysis, sometimes in the course of endocarditis can be observed Osler's nodules (painful and raised subcutaneous lesions on fingers or toes), Roth's spots (haemorrhagic areas with central retinal pallor) and Janeway lesions (reddish macules on the palms of the hands or soles of the feet) .

Felty syndrome

Splenomegaly is characteristic of this syndrome that develops in rheumatoid arthritis. Associated signs are pain and joint deformity. Loss of sensitivity or motor faculties. Rheumatoid nodules, palmar erythema, lymphadenopathy, and ulcers in the lower limbs.

Hepatitis

In this pathology splenomegaly can also be found (see hepatitis B and hepatitis C)

Histoplasmosis

Acute histoplasmosis usually causes splenomegaly, hepatomegaly, jaundice, fever, anorexia, and anemia.

Leukemia

The splenomegaly of modest or large entity is an early sign of both acute and chronic leukemia. In chronic granulocytic leukemia, splenomegaly can be painful, associated with hepatomegaly, lymphadenopathy, asthenia, general malaise, paleness, fevers, hard edema, hemorrhagic diathesis, weight loss, anorexia and abdominal pain, bone and joint disorders.

Malaria

Frequent symptom in malaria splenomegaly is usually preceded by the paroxysmal crisis characterized by shivering followed by high fever and then profuse sweating. Related effects may include headache, muscle pain and hepatomegaly. In benign malaria, these paroxysms alternate with periods of well-being. In severe cases of malaria, however, the patient may develop persistent high fever, orthostatic hypotension, convulsions, delirium.

tac abdomen: splenomegaly, see on the right
of the image a huge spleen with a turgid and tortuous vein

Mononucleosis

(Infectious). Frequent symptom of this pathology is the most evident splenomegaly during the second and third week of illness. Usually, it is associated with a triad of symptoms: sore throat, cervical lymphadenopathy and remitting fever with an evening peak of -38.3 "-38.9'C Sometimes hepatomegaly jaundice and maculopopular rash may occur.

Tumor of the pancreas

Pancreatic cancer can cause moderate or severe splenomegaly if the tumor mass compresses the splenic vein. Abdominal or back pain, anorexia, nausea and vomiting, weight loss, gastrointestinal bleeding, jaundice, pruritus, skin lesions, emotional lability, weakness and asthenia also appear.

Polycythemia vera

In the late stages of this pathology, the spleen can be greatly enlarged, leading to the appearance of early satiety, sensation of abdominal fullness, abdominal pain in the upper left quadrant or pleuritic chest pains. The clinical features, associated with splenomegaly, are varied and numerous. The patient may have intense red-colored oral mucosa, headache, dyspnea, heeling, dizziness, weakness and asthenia. It can also develop paresthesia of fingers and toes, mental disorders, hypacusia, alteration or doubling of vision, scotomata. Aumenlo of arterial pressure, intermittent diplopia. Other signs and symptoms include pruritus, urticaria, cyanosis steals, discomfort in the epigastric, weight loss, hepatomegaly with hemorrhagic diathesis.

Sarcoidosis

This ganulomatosis can cause splenomegaly and hepatomegaly often associated with a vague sensation of abdominal discomfort. Other signs and symptoms vary depending on the affected systems. Breaking the spleen. In this pathology, splenomegaly may be due to massive intraparenchymal hemorrhage. The patient may also have pain in the upper left quadrant of the abdomen, abdominal resistance and positive Kehr sign.

Thrombotic thrombocytopenic purpura

This pathology can give splenomegaly and hepatomegaly associated with fever, generalized purple, jaundice, pallor and vaginal bleeding with haematuria. Other signs may include asthenia, weakness, headache, pallor, abdominal pain arthralgias. Finally, the patient develops signs of neurological deterioration and renal failure. In pediatrics: in addition to the causes of splenomegaly described above, children may develop splenomegaly in case of histiocytosis, hemolytic hemolytic anemia, Gaucher disease, Niemann-Pick disease, hereditary spherocytosis, anemia, sickle cell disease and beta-thalassemia (Cooley's disease) ). The presence of splenic abscess is the most frequent cause of splenomegaly in immunocompromised children.

 

Gastroenterology