Myelofibrosis

Introduction Myelofibrosis (MF) is a kind of myeloproliferative disease caused by collagen hyperplasia in bone marrow hematopoietic tissue, and its fibrous tissue seriously affects hematopoietic function. Primary myelin is also called bone marrow. "Sclerosing disease", "unexplained myeloid metaplasia". The disease has different degrees of myelofibrosis, as well as extramedullary hematopoiesis mainly in the spleen, followed by liver and lymph nodes. The typical clinical manifestation is juvenile-erythrocytic anemia, and there are more teardrop-shaped red blood cells. The bone marrow puncture often shows dry pumping, the spleen is often swollen, and has different degrees of bone sclerosis.

Cause

[cause]

It has not been elucidated that some scholars believe that bone marrow fibrosis is caused by abnormal stimulation of hematopoietic stem cells, leading to fibrous tissue hyperplasia and even new bone formation, and bone marrow hematopoietic tissue involvement eventually leads to hematopoietic failure.

The main pathology of MF was changed to myelofibrosis and extramedullary hematopoiesis in spleen and liver lymph nodes. The occurrence of myelofibrosis occurs from the center to the outer periphery, starting from the proximal epiphysis of the spine, ribs, pelvis and femur and tibia, and then gradually spreading to the distal end of the extremities.

1) Early whole blood cell proliferation with mild myelofibrosis bone marrow cells showed varying degrees of proliferation. Red, granular, and megakaryocyte cell lines all proliferated, with megakaryocytes being the most obvious. The fat vacuoles disappeared and the reticular fibers increased, but the normal structure of the bone marrow was not affected. Hematopoietic cells account for more than 70%, and bone marrow stroma is mainly composed of soluble collagen.

2) Metaphase bone marrow atrophy and fibrosis stage fibrous tissue hyperplasia, accounting for 40% to 60% of bone marrow, hematopoietic cells account for 30%, megakaryocytes still proliferate. The trabecular bone is increased and thickened, and new bone formation is formed adjacent to the bone marrow. Each of the scattered hematopoietic regions is separated by a parallel bundle or spiral of matter formed by reticular fibers, collagen fibers, plasma cells, and stromal cells.

3) End stage of advanced myelofibrosis and osteosclerosis. It is mainly composed of bone trabecular bone hyperplasia, accounting for 30% to 40% of bone marrow. Both fibrous and osteosclerotic tissues were significantly proliferated, and the medullary cavity was narrow. Except for megakaryocytes, the hematopoietic cells of other lines were significantly reduced. In this period, the bone marrow matrix component is mainly composed of polymeric proteins, mainly expressing fibronectin, and the distribution of exogenous protein and TENASCIN is increased.

an examination

Related inspection

Serum total iron binding teardrop erythrocyte

Frontal lobe epilepsy is characterized by simple partial seizures, complex partial seizures, and secondary systemic seizures or mixed episodes of these episodes, usually occurring several times a day, and often occurring during sleep. Partial episodes of the frontal lobe can sometimes be confused with episodes caused by mental factors, which are common comorbidities.

1. Powerful reminder of frontal lobe epilepsy

(1) Usually the attack time is short.

(2) Complex partial seizures from the frontal lobe, usually accompanied by a slight episode of post-seizure confusion or non-occurrence.

(3) quickly caused secondary systemic seizures (frontal lobe epilepsy is more common than temporal lobe epilepsy).

(4) The symptoms of tonic or sporty posture are prominent.

(5) Commonly complicated gesture-type autonomic syndromes at the time of onset.

(6) When the discharge is bilateral, it often falls.

2. Types of seizures of seizure type frontal lobe epilepsy are described below, but multi-amount areas may be rapidly involved, and special seizure types may not be identified.

(1) Auxiliary exercise zone episodes: episodes in the auxiliary exercise zone, in the form of postural focal tonics accompanied by vocalization, speech pauses, and fencing postures. The patient's head and eyeball turned to the opposite side of the origin of epilepsy, the upper limb abduction on the contralateral side of the epileptic foci, the external rotation of the shoulder, and the elbow flexion, which looked like the patient was looking at his hand. The upper and lower extremities of the same side are abducted, and the distal end of the upper extremity is more pronounced than the distal end of the lower extremity. The clinical manifestation of this ipsilateral upper limb extending to the origin of the epilepsy is described as a "fencing posture."

(2) shackle back seizures: the form of seizures is complicated with part of the complex movement gesture autopsy, common autonomic nerve signs, such as mood and emotional changes.

(3) Forehead polar zone episodes: Forehead polar zone episodes include compulsive thinking or loss of initial contact and head and eye steering, possibly accompanied by evolution, including reverse motion and axial clonic tics and falls. Autonomic nerve sign.

(4) Seizures in the forehead area: The form of seizures in the frontal area is a complex part of the seizure accompanied by initial movement and gesture automatism, olfactory hallucinations and illusions, and autonomic signs.

(5) dorsolateral seizures: the form of seizures may be tonic or less common, accompanied by rotation of the eyes and head and speech stop.

(6) Island cover episodes: Characteristics of island cover attacks include chewing, salivation, swallowing, symptoms of the throat, cessation of speech, abdomen of the upper abdomen, fear, and autonomic nervous signs. Simple partial seizures, especially partial clonic facial muscle attacks, are common and may be unilateral. If a secondary sensory change occurs, numbness can be a symptom, especially in the hand. The illusion of taste is especially common in this area.

(7) Exercise cortex episodes: The main feature of motor cortex epilepsy is a simple partial seizure, which is based on the local anatomy of the affected side and the affected area. In the lower pre-Rolando area, there may be speech stop, vocalization or Speech disorder, often occurs in the lateral tonic-clonic movement or swallowing movement, systemic seizures. In the lateral fissure, partial movement episodes are not accompanied by progressive or Jacksonian episodes; especially in the contralateral upper limbs. When the central lobule is involved, there is a tonic movement in the ipsilateral foot, and sometimes a tonic movement occurs in the contralateral leg. Todd is common after the attack. Seizures originate precisely from the motor cortex, where the threshold for epileptic seizures is low and spreads to a wider area of epileptogenicity.

(8) Kojewnikow syndrome: There are currently two types of Kojewnikow syndrome, one of which is known as Rasmussen syndrome, which is an epilepsy syndrome included in childhood symptomatic epilepsy. The other type is a special type that represents a partial seizure of the lateral fissures of adults and children and is associated with different lesions in the motor area. Its main features are: 1 motility partial seizure, clear positioning; 2 late, usually in the site of somatic seizures occur myoclonus; 3 EEG presents normal background activity, there is a focal burst Abnormalities (spine and slow waves); 4 syndromes can occur at any age in childhood and adulthood; 5 often find the cause (tumor, vascular disease); 6 the syndrome does not show progressive (clinical , EEG or psychological, except those related to the evolution of pathogenic damage). This syndrome can be caused by mitochondrial encephalopathy (MELAS).

It should be noted that the anatomical origin of some patients with epilepsy is difficult to determine in a particular cerebral lobe, which includes symptoms associated with the anterior central and posterior central regions (excitation of the peripheral zone of the lateral fissure). This overlap to the adjacent anatomy is also seen in the island cover epilepsy.

The interictal scalp EEG trace of frontal lobe epilepsy can be: 1 no abnormalities; 2 sometimes background asymmetry, spikes or spikes in the forehead area; 3 spikes or slow waves (both unilateral or more common) Bilateral or seen on the unilateral majority of the brain). Intracranial tracing can sometimes distinguish between unilateral and bilateral lesions.

Different EEG manifestations of frontal lobe episodes may be associated with initial clinical symptoms. In a few cases, EEG abnormalities occur before the onset of clinical attacks, which can provide important localization information, such as: 1 frontal or multi-leaf, usually bilateral, low amplitude, fast activity, mixed spikes , rhythmic spike, rhythmic slow wave or rhythmic slow wave; 2 bilateral high amplitude single sharp wave, followed by diffuse low level wave.

According to the symptomology, intracranial electrode tracing can provide information about the temporal and spatial evolution of the release; positioning is sometimes difficult.

The presence of one of the following six characteristics can be diagnosed as frontal lobe epilepsy:

1. Systemic tonic - immediate loss of consciousness after the onset of clonic convulsions.

2. At the beginning of seizures, the head and eyes turn to the opposite side, followed by systemic convulsions, loss of consciousness after the onset, often suggesting that the epileptogenic focus is located in the first 1/3 of the frontal lobe.

3. The initial head and eye turn to the contralateral side of the lesion, the consciousness is clear and gradually unconscious, and then the total loss of consciousness and systemic seizures suggest that the epileptogenic focus originates from the middle part of the frontal lobe.

4. The posture is a posture movement of a certain part of the body, such as the contralateral arm is raised and raised, the ipsilateral arm is extended downward and the head is turned to the opposite side of the lesion, suggesting that the epileptogenic focus is located on the inner side of the middle part of the frontal lobe.

5. Often showing no emotions, or short-term pauses, confusion, and gaze. Then a generalized seizure occurred.

6. Seizures may have an episode or an autopsy after the onset, similar to temporal lobe epilepsy. Interictal SPECT and PET can confirm local hypoperfusion or low metabolism in the brain, while SPECT in the episode often shows high perfusion of the frontal cortex, which contributes to the localization of epileptic foci.

At present, a consensus has been reached that 80% to 90% of patients with epilepsy diagnosed with MRI have a good effect after epileptic surgery. For non-focal epilepsy with no positive findings on MRI, postoperative outcomes are not ideal.

Differential diagnosis

The myelin should be differentiated from the following diseases: 1 Chronic myeloid leukemia: Both can have giant spleen, the number of white blood cells increases, and the peripheral blood appears granulocyte proliferation like neutrophils and late myelocytes, but the age of chronic granules is lighter. The white blood cell count is often more than 100,000/mm3, and there are fewer myelocytes in the blood. The deformity of red blood cells is not similar to that of myelin. Leukocyte alkaline phosphatase activity is reduced or eliminated and the ph' chromosome is distinguishable from myelin. 2 The disease must be distinguished from low-proliferative acute leukemia and other diseases that cause juvenile-erythrocytic anemia. Secondary myelin can be diagnosed from clinical manifestations or special examinations. Sometimes multiple sites, multiple bone marrow smears and biopsy are needed to exclude secondary myelin.

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