Acute Lymphoblastic Leukemia in a Child Presenting Primarily with Priapism.

Priapism is an uncommon presentation in children and adults with a chronic myeloid type of leukemia. Its association is rarely found in an acute lymphoblastic type of leukemia (ALL). Timely management is important to prevent irreversible complications. We report a case of ALL with presenting complaint of priapism. Copyright:

INTRODUCTION

Priapism is a prolonged full or partial penile erection lasting for more than 4 h in the absence or after the end of sexual stimulus. Prolonged priapism extending beyond 24–48 h may result in fibrosis and injury to erectile tissue which subsequently may result in erectile dysfunction. Hence, prompt diagnosis and management is necessary. It is seen more commonly during the fifth decade of life.[12] Among children, the common causes of priapism are sickle cell disease followed by trauma.[3] Leukemia is a rare cause of priapism in children, out of which around 50% are associated with chronic myeloid leukemia (CML).[4] There are only few case reports of leukemia associated with priapism. Association of acute lymphoblastic type of leukemia (ALL) with priapism is even rarer.

CASE REPORT

A 10-year-old male child admitted with complaints of fever, headache, dizziness, pallor, and painful persistent erection of the penis. There was no history of sexual arousal, drug intake, and injury to the genitalia. There was no history of pain abdomen, vomiting, rashes, bleeding from any site, blood transfusion, and similar episode in the past. On examination, the child was oriented; pallor was present; and cervical, axillary, and inguinal lymph nodes were enlarged. The penis was erect, firm, and tender; the glans was not that turbid compared to the body/shaft; and there were prominent superficial veins. The liver was 3 cm in size, and there was no splenomegaly. Rest of the other systems were normal. Investigations revealed hemoglobin of 5.0 g/dL; total leukocyte count of 693,000/mm3; platelet count of 40,000/mm3; serum calcium of 8.4 mg/dL; findings of liver enzymes such as serum glutamic-pyruvic transaminase of 63 IU/dL, serum glutamic oxaloacetic transaminase of 90 IU/dL, uric acid of 5.3 mg/dL, serum lactate dehydrogenase of 2343 IU/dL; and blood urea and serum electrolytes were within normal limits. Peripheral smear showed marked leukocytosis with 95% blast cells. Chest X-ray showed mediastinal widening. Bone marrow aspirate was suggestive of ALL. Flow cytometry was positive for CD19, CD20, human leukocyte antigen-DR, CD34, CD79a, and CD33 suggestive of B-cell ALL. Color Doppler ultrasonography suggested low-flow priapism. This suggested an ischemic type of priapism. Corporal aspiration followed by phenylephrine irrigation achieved partial detumescence with reduction of pain. The child was given intravenous fluids and injections ceftriaxone and amikacin. Injection dexamethasone was started for leukocytosis, and injection NaHCO3 and tablet allopurinol were started to prevent tumor lysis syndrome and the child was monitored for the same. Packed cell transfusion was given. Priapism resolved after 2 days of therapy. After 2 days of admission, the child became confused and developed altered sensorium. On examination, the child was drowsy and had ataxia. There were an increased tone in all the four limbs and brisk deep tendon reflexes with extensor plantar response. There was no cranial nerve involvement. Hence, the possibilities of central nervous system (CNS) leukemia, intracranial hemorrhage, or CNS infarct due to hyperleukocytosis were kept. The child had respiratory failure due to CNS involvement. The child was taken on the ventilator and steroids and allopurinol were continued, but unfortunately, he succumbed to death.

DISCUSSION

About half of the pediatric patients with priapism are found to have CML although it has been reported in children with acute myeloblastic leukemia and ALL also.[4] The exact prevalence of priapism is not known; however, it is found to be rare in children. The common causes of priapism in children are enumerated in Table 1. Nearly 20% of priapism is the result of hematological abnormalities. In adults with leukemia, priapism is found to be associated with 1%–2% of cases only. Priapism among leukemic patients has been reported in CML, chronic leukemoid leukemia, and T-cell leukemia.[4] We have diagnosed B-cell type of leukemia in the present case with priapism.

Table 1

Causes of priapism in children

Ischemic type

SCD, G6PD, CDA, thalassemia

ALL, CML

HSP

Rhabdomyosarcoma, testicular tumors

Infections - Sepsis, malaria

Snake/spider/scorpion bite

TPN

Nonischemic type

Trauma

Fabry disease

Drugs

SCD: Sickle cell disease, ALL/CML: Acute lymphocytic leukemia/chronic myeloid leukemia, HSP: Henoch–Schonlein purpura, TPN: Total parentral nutrition, CDA: Congenital dyserythropoetic anemia, G6PD: Glucose 6 phosphate dehydrogenase deficiency

Priapism has been divided into three types, namely ischemic (low flow, veno occlusive), stuttering (intermittent, recurrent ischemic), and nonischemic (high flow, arterial). In leukemia, priapism is of ischemic type; it develops as a result of venous congestion of the corpora cavernosa resulting from mechanical pressure on the abdominal vein by organomegaly and sludging of leukemic cells in the corpora cavernosa and dorsal vein of the penis or with infiltration of the sacral nerves with leukemic cells.[5]

The most common type of priapism in children is ischemic type which is very painful. It is further differentiated from nonischemic type by doing intracavernosal blood sampling which shows acidosis (pH <7.25), hypercarbia (PCO2>90), and decreased oxygen tension.[6] Furthermore, differentiation can be made by local examination of the genitalia; in ischemic type, the shaft of the penis is turbid, whereas in nonischemic type, whole of the penis is rigid including the glans and shaft. It is important to differentiate the two types, as in the ischemic type of priapism, if treatment is not started within 24–48 h, irreversible cellular damage and fibrosis can occur and may result in impotency.[7] In our patient, there was high lymphocytic load, and the CNS was involved. On examination, compared to the shaft which was rigid, the glans was found to be flaccid [Figure 1]; it was extremely painful, blood aspirated was bright red,[8] and gas analysis of the aspirated blood revealed pH – 7.14, PCO2– 96, and PO2– 45.

Figure 1

Priapism

Management includes assessing the type of priapism and achieving detumescence [Figure 1]. Ischemic priapism has to be corrected within 24–48 h of the onset to prevent complications. Opiate analgesia may achieve detumescence in ischemic priapism.[9] Physical exercise, ejaculation, and urination can be used as the first aid methods in adults and grown children.[10] The next step includes lavage of corpus cavernosa with normal saline (1 ml/kg) followed by irrigation with sympathomimetic.[11] In the case of refractory priapism, methods used are (a) aspiration – blood is aspirated by inserting needle into the cavernosa and (b) T shunt formation – types of shunts are – distal shunts; for example, cavernoglanular, percutaneous (Winter's), and open (Al Ghorab shunt) and the other type is proximal shunts; for example, quackles, cavernospongiosal, and cavernovenous (Grayhack shunt).[12] Distal shunts can be tried initially, but maintenance of detumescence is not usually good with them; if not maintained, then the proximal type of shunts can be used. As the underlying etiology for priapism in leukemia is known, reduction of cell counts immediately using leukapheresis and by starting chemotherapy may help relieve the symptoms. Adult case reports suggest that surgery should be considered earlier for management, but pediatric case reports warrant conservative management primarily among leukemia patients as it is associated with less incidence of erectile dysfunction.[13] In our case, combined systemic and surgical intervention successfully achieved detumescence without shunt creation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Nil.

Conflicts of interest

There are no conflicts of interest.