The post-mortem diagnosis of vasocclusive crisis in sickle cell disease
Several studies highlight that acute chest syndromes, cerebrovascular events, splenic dysfunction or sequestration, and aplastic crises are the main causes of sudden death, although any presentation of sickling can lead to death if one of these more serious complications then arises.
We hereby present two cases: a female child and an adult male (
|Parameters||Case 1||Case 2|
|Resident of||Maharashtra, India||Maharashtra, India|
|Presentation||Fever, seizure one episode||Fever with chills, headache|
|Past history||Not significant||Not significant|
|Autopsy performed at||Alibaug Civil Hospital, Mumbai||Rajawadi Hospital, Mumbai|
|Autopsy finding||Hepatosplenomegaly with yellow discoloration of omentum||Hepatosplenomegaly with congestion in other organs|
|Provisional cause of death at autopsy||Pyrexia with hepatosplenomegaly to rule out chronic malaria/hepatitis/leptospirosis||Pending for histopathology examination|
|Viscera received at||Department of Pathology at Grant Government Medical College and Sir JJ Group of Hospitals, Maharashtra||Department of Pathology at Grant Government Medical College and Sir JJ Group of Hospitals, Maharashtra|
|Microscopy||Spleen, liver and brain showed congested blood vessels with sickle-shaped RBCs (
||All organs showed congested blood vessels with sickle shaped RBCs (
|Final cause of death||SCD with the possibility of vaso occlusive crisis in a child||Pulmonary tuberculosis in a case of SCD with the possibility of vaso occlusive crisis|
SCD is remarkable for its clinical heterogeneity. There is a dearth of data on SCD in India compared with other countries in Africa and America. The clinical profile of sickle cell patients in Central India is less severe compared that of African countries and is characterized by delayed presentation, pauci-symptomatic cases, less frequency of vaso occlusive crisis, and low mortality. Therefore, most of the patients remain undiagnosed.
We are reporting these rare cases of sudden death in a previously undiagnosed SCD in a female child and an adult male of Indian origin with no remarkable medical histories.
Our patients had a very short clinical history of illness of 24 hours. They had no clinical evidence of acute hemolysis, abdominal pain, breathlessness, or anxiety to suggest sickle crisis. The recognized putative factors for sickle cell crisis include infection, fever, excessive exercise, anxiety, and hypoxia,
The microscopic findings seen in both cases revealed congested blood vessels packed with sickled red blood cells (RBCs) in multiple organs. The incidental finding of necrotizing granulomatous inflammation consistent with tuberculosis was noted in the adult male. As splenomegaly was noted in the autopsy notes of both our cases, the possibility of other hemoglobinopathies, like double heterozygous HbS and HbC, could not be ruled out by means of histology; therefore, the broad diagnostic possibility of SCD was considered.
Hemoglobin electrophoresis is the most common method used to diagnose SCD at autopsy. However, Thogmartin et al.,
We conclude that limited clinical history, inadequate evaluation of pathological findings at autopsy, and a lack of laboratory resources (electrophoresis) are major shortcomings in the diagnosis of SCD in medicolegal cases and cases of sudden death. Therefore, autopsy pathologists should be aware of this possibility in such cases. However, precise histopathological examination of tissue in the absence of other ancillary techniques serves as an important tool in diagnosing SCD at autopsy.