Leukostasis is associated with people who suffer from bone and blood disorders and is very common among people suffering from acute myeloid leukemia or chronic myeloid leukemia. Leukostasis is a pathologic diagnosis that inhibits efficient flow to the microvasculature of the body. Continued and untreated leukostasis presents respiratory and neurological distress simultaneously and is a medical emergency, with untreated patient mortality rates reaching a minimum of 20 and a maximum of 40 percent.. As stated before, these counts are critical and associated with Leukemias.

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China, E-mail: moc. The article may be redistributed, reproduced, and reused for non-commercial purposes, provided the original source is properly cited. This article has been cited by other articles in PMC. It may present with a variety of symptoms secondary to leukostasis, a syndrome caused by the sludging of circulating leukemic blasts in the microvasculature.

Adequate measures for managing this medical emergency include hydration, cytoreduction, prevention of tumor lysis and, rarely, leukapheresis in cases complicated by leukostasis and hyperviscosity syndrome. The present study reports a case series of five patients with hyperleukocytic leukemia. In addition, a review of the literature with regard to the incidence, pathophysiology, clinical manifestations and management of this laboratory abnormality is included.

This study demonstrated that the central nervous system and lungs are the most common sites for leukostasis, and that emergency cases require aggressive treatment. Keywords: hyperleukocytosis, leukostasis Introduction In patients with acute lymphocytic leukemia ALL or acute myeloid leukemia AML , the clinical presentation is usually non-specific, including fatigue, fever or bleeding. However, certain patients with acute leukemia present with clinical signs of hyperleukocytosis 1.

Leukostasis is rapidly progressive and fatal in the majority of patients 2 , 3. The current study presents five patients with hyperleukocytosis. Cases 1, 2 and 3 were AML with induced leukostasis syndrome, while cases 4 and 5 were ALL without leukostasis syndrome. Patients provided written informed consent. Initial laboratory tests revealed a WBC count of The patient was transferred to the Department of Haematology due to suspected acute leukemia. On physical examination, the patient had a temperature of A chest computed tomography CT scan showed a diffuse high-density mass shadow in the two lungs.

Secondary laboratory tests revealed a WBC count of Bone marrow smear and immunophenotyping studies showed AML [acute monocytic leukemia subtype M5 ], according to the French-American-British classification on the basis of morphological features, and histochemical staining of cells with peroxidase and esterase 4. Blood gas analysis revealed the following: pH, 7. Hydroxyurea 1.

Three days following transferral to the Department of Haematology, the patient succumbed to the disease. Initial laboratory studies revealed a WBC count of Bone marrow smear revealed AML M5. An emergency head CT scan showed cerebral infarction. The patient succumbed to the disease 5 h later.

Bone marrow smear showed AML M5. On day three, the patient experienced a severe headache and left arm numbness, with projectile vomiting, Laboratory tests revealed a WBC count of An emergency head CT scan showed cerebral hemorrhage.

The patient succumbed to the disease 8 h later. On physical examination, the patient demonstrated a fever temperature, Sternal tenderness was also evident. Dexamethasone 15 mg per day was administered by intravenous infusion for five days, to induce leukoreduction. The patient acquired complete response after 58 days.

Bone marrow smear showed ALL, and immunophenotyping studies showed acute B-lymphocytic leukemia. Discussion Several cases of hyperleukocytosis have previously been reported. Brain CT scan showed multiple intracerebral haemorrhages, while chest CT showed a parenchymal right basal consolidation and diffuse infiltration. Following emergency treatment and combination chemotherapy, the male achieved complete remission 1.

Brain CT showed multiple areas of parenchymal haemorrhage with intraventricular hemorrhage and subdural hematoma along the posterior falx. However, the male was declared brain dead and care was withdrawn. In the present study, the two cases of acute lymphocytic leukemia were not complicated by leukostasis syndrome, possibly due to lower WBC counts.

All seven patients were treated with leukocytapheresis. Further treatment was based on chemotherapy alone, and leukapheresis treatment was terminated to avoid interference with the chemotherapy.

Two of the patients critically ill on admission to hospital succumbed during the first week following diagnosis of leukaemia, while five patients acquired complete remission 6. In the present study, the two patients with acute myeloid leukaemia complicated with leukostasis syndrome abandoned all treatment and were discharged from hospital.

While case one succumbed at hosptital during the first week following the diagnosis of leukaemia. Therefore, leukostasis syndrome is an emergency case.

This laboratory abnormality can cause severe mortality by inducing one or more of the following: Leukostasis, tumor lysis syndrome and disseminated intravascular coagulopathy DIC 8.

Hyperleukocytosis has significant prognostic implications with or without one of the clinical complications mentioned above. Hence, if hyperleukocytosis occurs in lymphatic leukemias, it does so at markedly higher WBC counts 9. The pathophysiology of leukostasis is not clear.

There are two leading theories. The rheological theory relates to the principle that blood viscosity is a function of two factors, the deformability of individual cells and the volume of the cell fraction in the blood.

Blasts are less deformable than mature WBCs. For elevated WBC counts, the high fractional volume of leukocytes leukocrit results in increased blood viscosity. As a result, the non-deformable blasts can occlude microvessels and reduce flow in the vessels with marginally larger caliber.

Both mechanisms lead to vascular obstruction, which induces tissue hypoxia. Leukostasis leads to vascular obstruction, and the central CNS and lungs are the most common sites for this The CNS symptoms may include confusion, dizziness, headache, tinnitus, blurred vision, somnolence, stupor, delirium, coma and ataxia.

On examination, focal deficits may be elicited and retinal hemorrhages may be present CT scan or magnetic resonance imaging of the head may reveal intracranial hemorrhage. Respiratory symptoms include dyspnea, tachypnea and hypoxemia, with the presence of auscultatory rales.

A chest X-ray or a CT scan often will show bilateral interstitial or alveolar infiltrates 3. In AML, the majority of studies have found that hyperleukocytosis is a poor prognostic factor AML patients with hyperleukocytosis have demonstrated lower complete remission rates, disease free survival and overall survival, as well as high rates of early mortality.

In ALL, there are consistent data regarding the poor prognosis of hyperleukocytosis. It is unknown whether the prognostic effect of hyperleukocytosis is a result of high tumor burden or whether those leukemias with high WBC counts are distinct entities with different pathophysiology and clinical characteristics.

It may be, for example, that hyperleukocytosis is an expression of a molecular change, such as the FLT3-ITD mutation in AML, and that the molecular aberration itself is responsible for the poor prognosis rather than the actual WBC count.

In several leukemias, there is an association of hyperleukocytosis with specific subtypes of the disease. For example, several studies have linked hyperleukocytosis to monocytic differentiation subtypes, particularly M4Eo and M5a in AML, and M3v in acute promyelocytic leukemia, while in ALL there is an association with t and t The management of hyperleukocytosis includes intensive supportive care and cytoreduction.

Supportive care consists of prevention of tumor lysis syndrome by aggressive hydration and allopurinol, and respiratory support as required. The cytoreduction may be achieved by leukapheresis, induction chemotherapy and hydroxyurea.

A critical problem is that if the WBC counts are not reduced prior to induction therapy, tumor lysis syndrome and DIC may be aggravated with the induction treatment. In AML, leukapheresis and hydroxyurea are used to cytoreduction; while in ALL, leukapheresis and dexamethasone are used In the present case series, all five patients presented with hyperleukocytic leukemia. The reason is that myeloblasts are larger in size than lymphoblasts and lymphocytes.

The sites of leukostasis were the lungs in case 1 and the CNS in cases 2 and 3, which were both invalid. Hence, physicians should begin aggressive treatment for leukostasis when the first respiratory or neurological symptoms or sign appear in a leukemic patient with hyperleukocytosis. This suggested that the poor prognosis of hyperleukocytosis may be linked to the monocytic differentiation subtypes in AML, while in ALL there is an association with t This is consistent with previous reports in the literature 3 , 12 , In conclusion, hyperleukocytosis has a significant prognostic implication.

Leukostasis syndrome frequently arises in hyperleukocytosis, with common sites including the lung and CNS. Leukostasis syndrome is an emergency case with a high rate of early mortality, therefore, the management must be aggressive. The overall aim is to reduce early mortality, however, the pathophysiology of leukostasis remains unclear and future studies are required.

References 1. Severe hyperleukocytosis and multifocal intracranial haemorrhage: not always a fatal outcome. Int J Hematol. Outcome of acute myeloid leukemia patients with hyperleukocytosis in Brazil. Med Oncol. Reversal of leukostasis-related pulmonary distress syndrome after leukapheresis and low-dose chemotherapy in acute myeloid leukemia. J Clin Oncol. Proteomic analysis of human acute leukemia cells: insight into their classification. Clin Cancer Res. Cerebral hemorrhage due to hyperleukocytosis.

J Emerg Med. Hyperleukocytosis and leukocytapheresis in acute leukaemias: experience from a single centre and review of the literature of leukocytapheresis in acute myeloid leukaemia. Hyperleukocytic leukemias and leukostasis: a review of pathophysiology, clinical presentation and management.


Hyperleukocytosis: A report of five cases and review of the literature

China, E-mail: moc. The article may be redistributed, reproduced, and reused for non-commercial purposes, provided the original source is properly cited. This article has been cited by other articles in PMC. It may present with a variety of symptoms secondary to leukostasis, a syndrome caused by the sludging of circulating leukemic blasts in the microvasculature.



Grogami Hyperleukocytosis, leukostasis and leukapheresis: practice management. In addition, leukemic blasts have a higher rate of oxygen consumption and thus may compete with tissue cells leukosfasis areas of obstructed flow. Definitive treatment of pulmonary leukostasis is still controversial; however, early detection and treatment by cytoreduction may improve outcomes. You can help by adding to it. High WBC especially percentage and absolute number of circulating blasts. Br J Haematol 98 2: Technical matters Venous access is usually the rate-limiting step in any apheresis procedure. Twenty to forty percent peukostasis patients diagnosed with hyperkeuckocytosis die within the first week of symptom presentation.


Leukostasis and hyperleukocytosis


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